2 AinShamsLectureNotes 2020 CHS

Part II
Congenital
Heart
Surgery (CHS)
Ain Shams Lecture Notes

in
Cardiovascular & Thoracic Surgery
Ezzeldin A. Mostafa, MD, PhD, MBA

Professor of Cardiovascular & Thoracic Surgery
Ashraf A. Elmidany, MD, PhD

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Ain Shams Lecture Notes in
Cardiovascular
&
Thoracic
Surgery
Volume II: Congenital Heart Surgery (Second Edition)
Department of Cardiovascular & Thoracic Surgery
Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Ashraf A. Elmidany, MD, PhD
Associate Professor of Cardiovascular & Thoracic Surgery
Department of Cardiovascular & Thoracic Surgery
Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Copyright Information
Ain Shams Lecture Notes in Cardiovascular & Thoracic Surgery
Volume II: Congenital Heart Surgery (Second Edition)
Copyright © 2016 by Elnasr Publishing Co. All rights reserved. Printed in Egypt. Except as permitted
under the Egyptian Copyright Act of 1976, no part of this publication may be reproduced or distributed in
any form or by any means, or stored in a data base or retrieval system, without the prior written
permission of the publisher.
ISBN 978.977.90.5818.4
NOTICE
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Ain Shams Lecture Notes in Cardiovascular & Thoracic Surgery
Part I: Congenital Heart Surgery
To My Students
You will remember
some of what you hear,
much of what you read,
more of what you see,
and
almost all of what you experience and understand fully.

‘Happiness comes when you
believe in what you are doing,
know what you are doing,
and
love what you are doing’’


Tell me.....and I will forget
Show me.....and I may remember
Involve me.....and I will understand

Confucius, 450 BC

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Symbols, Abbreviations & Acronyms
This is the list of abbreviations and acronyms which occur frequently in the text. Those less commonly
used are explained where they occur.
General Abbreviations & Acronyms
1
General Abbreviations & Acronyms (Cont’d)
Symbols
 ≈ approximately  ê decreased 
§ cross-reference Dx Diagnosis
° degrees Hx History
® Trade name é increased 
+ ve positive  è leads to or results in
± with/without M:F Male to Female ratio
< less than ò Female
> more than ô Male
− ve negative  Tx Treatment
≤equal or less than α alpha
≥ equal or more than β beta
DDx or DD differential diagnosis δ delta
Δ triangle
μ mean
2
Specific Abbreviations & Acronyms
ABG arterial blood gas DO2 oxygen delivery
ACE angiotensin-converting enzyme DSWI deep sternal wound infection
AChR acetyl cholinesterase receptor DVT deep vein thrombosis 
ADH antidiuretic hormone E/M electron microscopy
ADP adenosine diphosphate EBV Epstein–Barr virus 
AF atrial fibrillation or AFib
ECF extracellular fluid
ECMO extracorporeal membrane oxygenation
AMI acute myocardial infarction 
EEG electroencephalogram 
ANS autonomic nervous system 
EKG electrocardiogram 
AP anteroposterior 
EMG electromyogram 
APC antigen presenting cell  EPO erythropoietin
APTT activated partial thromboplastin time ERT emergency room thoracotomy
ARDS acute respiratory distress syndrome ET endotracheal 
ARF acute respiratory failure
EtOH ethanol (alcohol) 
ARI acute renal insufficiency
ATP adenosine triphosphate EUS endoscopic ultrasound 
bid twice a day (bis in die)  EVLW extravascular lung water
FB foreign body or feedback
BiPAP biphasic positive pressure ventilation 
FBC full blood count 
BP blood pressure 
Fe iron
BSA bovine serum albumin  FEV1 forced expiratory volume in 1 second
BUN blood urea nitrogen
C/O complaint of FFP fresh frozen plasma 
FFR fractional flow reserve
Ca2+ calcium  FiO2 fraction of inspired oxygen
CAVH continuous arteriovenous hemofiltration 
FVC forced vital capacity g gram/s
CBC complete blood count  GFR glomerular filtration rate
CDC Centers for Disease Control and Prevention GI gastrointestinal
  HACEK Hemophilus, Actinobacillus,
CHF congestive heart failure  Cardiobacterium, Eikenella, Kingella
CLL chronic lymphocytic leukemia  HAR hyperacute rejection
cm centimeter/s HCV hepatitis C virus
CMR cardiac magnetic resonance  HIT heparin-induced thrombocytopenia
CMV cytomegalovirus or controlled mechanical HITT heparin-induced thrombocytopenia and
ventilation  thrombosis
CN cyanide  HLA human leukocyte antigen
CNS central nervous system HR heart rate
CO cardiac output hr hour
COPD chronic obstructive pulmonary disease  HTLV human T-lymphotrophic virus
IABP intra-aortic balloon pump
CPAP continuous positive airway pressure  ICD implantable cardioverter-defibrillator or
CPB cardiopulmonary bypass  International Classification of Diseases
CPR cardiopulmonary resuscitation  ICU intensive care unit
CRI chronic renal insufficiency IM intramuscular 
CRP C-reactive protein  INPV intermittent negative pressure ventilation
CSF cerebrospinal fluid  INR international normalized ratio 
CT computed tomography  Int intermediate 
CTA computed tomography angiogram IPPV intermittent positive pressure ventilation
CVA cerebrovascular accident ITA internal thoracic artery
CVP continuous venous pressure IV intravenous
CVVH continuous venovenous hemofiltration IVC inferior vena cava 
CXR chest X-ray IVUS intravascular ultrasound
DIC disseminated intravascular coagulation JVP jugular venous pressure 
DLCO diffusing capacity of the lung for carbon K potassium
monoxide kg kilogram/s L liter/s
DMSO dimethyl sulfoxide solution
L milliliter/s m
III
L. left-sided PAPVC partial anomalous pulmonary venous
LA left atrium/atrial  connection   
LAO left anterior oblique   PAWP pulmonary artery wedge pressure
LAP left atrial pressure   PDA posterior descending artery or patent ductus
LAP mean left atrial pressure  arteriosus
LASER is an acronym for Light Amplification by PDGF platelet-derived growth factor 
Stimulated Emission Radiation. PEA pulseless electrical activity 
LFT liver function test  PEEP positive end-expiratory pressure 
LMS left main stem  PET positron emission tomography 
LMWH low molecular weight heparin  PFO patent foramen ovale 
LN lymph node  PGE1 prostaglandin E1 
LPA left pulmonary artery  PICU pediatric intensive care unit 
LSV long saphenous vein Plts platelets
LSVC left superior vena cava PNS peripheral nervous system
LV left ventricle  po by mouth (per os) 
M/E microscopic examination POBA plain old balloon angioplasty 
MAO monoamine oxidase   PPI proton pump inhibitor 
MAP mean arterial pressure   ppo predicted postoperative 
MAPCA major aortopulmonary collateral artery  prn pro re nata (as required) 
MCV mean corpuscular volume  PS pulmonary stenosis or pressure support
MDT multidisciplinary team  PSV pressure support ventilation
mg milligram/s  PT prothrombin time 
MG myasthenia gravis  PTCA percutaneous transluminal coronary
MI myocardial infarction angioplasty
min minute/s m PTE pulmonary thromboembolism 
MMF mycophenolate mofetil PVC polyvinyl chloride 
mmol millimole/s PVR peripheral vascular resistance 
MMV mandatory minute ventilation PVRI pulmonary vascular resistance index 
mPAP mean pulmonary artery pressure qid four times a day (quater in die) 
MRA magnetic resonance angiography R. right-sided
MRI magnetic resonance imaging RA right atrium/atrial 
MRSA meticillin-resistant Staphylococcus aureus
RAAS renin–angiotensin–aldosterone system 
ms millisecond/s
MVO2 mixed venous oxygen saturation RAO right anterior oblique  
RAP right atrial pressure  
MVV maximal voluntary ventilation
N/E naked eye appearance RBB right bundle branch  
Na sodium RBC red blood cell   
NBM nil by mouth RCT randomized controlled trial
NG nasogastric RPA right pulmonary artery
NIBP non-invasive blood pressure RSPV right superior pulmonary vein 
NIPPV non-invasive intermittent positive pressure RSV respiratory syncytial virus 
ventilation RV right ventricle 
NO nitric oxide S. & S. symptoms and signs
nocte at night s/l sublingual 
NPO withold food and drink by mouth (nil per os) SA sinoatrial
NSAID non-steroidal anti-inflammatory drug SAM systolic anterior motion 
NYHA New York Heart Association
SaO2 oxygen saturation of arterial blood 
O/E on examination
OCP oral contraceptive pill SBT spontaneous breathing trials 
od once a day (omne in die) SC subcutaneous 
OR operating room or odds ratio SCBU special care baby unit 
pa per annum SIMV synchronized intermittent mandatory
PA pulmonary artery or posteroanterior or ventilation 
physician assistant SIRS systemic inflammatory response syndrome 
PAH pulmonary arterial hypertension
SLE systemic lupus erythematosus 
PAN polyarteritis nodosa 
SNP sodium nitroprusside 
PAP pulmonary artery pressure
IV
SPECT single positron emission computed U unit/s 
tomography U/S ultrasound 
SR survival rate U&E urea and electrolytes 
SSEP somatosensory evoked potential  URTI upper respiratory tract infection 
SVC superior vena cava  VATS video-assisted thoracic surgery
SvO2 mixed venous oxygen saturation VAVD vacuum-assisted venous drainage
SVR systemic vascular resistance  VC vital capacity 
SWI superficial wound infection  VF ventricular fibrillation 
TAPVC total anomalous pulmonary venous vit vitamin 
connection   VQ ventilation-perfusion 
TEE transesophageal echocardiography   VSD ventricular septal defect 
TEG thrombelastography   VT ventricular tachycardia, VTach 
TIA transient ischemic attack WCC white cell count
tid three times a day (ter in die)  WL window level  
TIVA total intravenous anesthesia  WW window width 
TLC total lung capacity  XM cross match
TOF tetralogy of Fallot or tet yr(s) year (s)
TOS thoracic outlet syndrome  
TRAM transverse rectus abdominis
myocutaneous
TTE transthoracic echocardiography 
V
1 General Cosiderations
2 Acyanotic, without Shunt, Left Side
3 Acyanotic, without Shunt, Right Side
4 Acyanotic, with L®RS
5 Cyanotic with PBF
6 Cyanotic with ¯ PBF
7 Cyanotic with Pulmonary Hypertension
ezzeldinmostafa.com
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Congenital Heart Surgery Spring 2016

Dr. Ezzeldin Mostafa emostafa@med.asu.edu.eg 1. General Considerations
www.ezzeldinmostafa.com Page 1 of 39
1 General Considerations
Table of Contents Blue, well-perfused baby .................................. 10

Happy Tachypnea - too much pulmonary flow . 10
1 General Considerations .......................... 2 Pink, shocky infant ........................................... 10
Definitions & Nomenclatures ................................. 4
Systematic ECHO exam .................................. 15
Segmental Anatomy & Nomenclature ................ 4
Treatment Considerations ................................... 19
Terminology ........................................................ 7
Palliative procedures for CHDs ........................ 21
Sequential Segmental Analysis .......................... 7
Pulmonary artery banding & the Norwood
Morphology of Cardiac Segments ...................... 4
procedure ......................................................... 24
Fetal circulation .................................................. 7
Atrial septostomy .............................................. 25
General Pathologic Considerations ....................... 7
Atrial switch operation (ASO) ........................... 26
Classification of congenital heart disease .......... 7
Other palliative procedures .............................. 26
Clinical Picture & Diagnostic Considerations ......... 9
Pacemaker use in pediatrics ............................ 27
Diagnosing CHD in the Sick Infant ..................... 9
Implantation In Children ................................... 27
Shock - Not enough systemic flow ..................... 9
Pediatric Cardiac Mechanical Support ............. 30
Blue, shocky infant ............................................. 9
Residuae, Sequalae & Complications .............. 33
Happy Cyanosis - not enough pulmonary flow ... 9
Prognostic Considerations................................... 34
Gray baby ........................................................... 9
Sick Cyanosis - pulmonary venous congestion 10
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Unit Objectives General Cardiology, Segmental

• To review the surgical anatomy of the cardiovascular system and Anatomy & Nomenclature
surgical significance of eachpart. Normal Cardiac Anatomy - Anderson
• Structural functional relationships and review anatomy and Ch2
histology and overall anatomy
https://sites.google.com/a/pedscards.c
• To review the anatomical correlation with the investigations like the
EKG, ECHO (TTE &TEE), ANGIO, and different imaging studies to om/pedscards-com/cardiology-
orient the surgeon’s perioperative management. notes/normal-cardiac-anatomy
• To know my way to understand the embryology of the CVS and its Nomenclature- Moss and Adams
clinic-physiologic classification of the congenital heart disease
(CHD)
om/pedscards-com/cardiology-
notes/ma02-classification--
terminology
Anderson Terminology Algorithm
Terminology - Anderson Ch1
Lai Echocardiography
om/pedscards-
com/echocardiograms/segmental-
anatomy--nomenclature-lai3
Surgical Eponyms Link
Pediatric Cardiology Milestones -
Timeline
Learning Objectives:
By the end of this chapter the student should Know:
• Surface anatomy
• Cardiac chambers
• Right atrium
• Right ventricle
• Left ventricle
• Conduction system
• Cardiac valves
• Descriptive variables
Emberyology
Refer to http://embryology.med.unsw.edu.au/
Or youtube
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Definitions & Nomenclatures
Segmental Anatomy & Nomenclature

Van Praagh Approach (Boston)
3 main segments to the heart

-veins/atria
-ventricles
-GAs
2 main connecting segments
-AV canal = AV valves
-conus (aka infundibulum) = connection bn GA and ventricles
-circumferential subpulm myocrdium shaped like a
cone or funnel (internal shape)
-pulm infundib separates TV and PV
-subAo infundibulum = only the conal septum- the
myocardium separating the LV and RV
-there is NO free wall part, so there is fibrous continuity bn Ao
vlv (L and noncor leaf) and the ant leaflet of the MV ==>
"MV-Ao continuity" (in DORV, there is a full infundib, so --> MV-
Ao discontinuity)
Approach to Describing Cardiac in 10 Steps:
Van Praagh Andersonian

Nomenclature Nomenclature
1. Thoraco-abdominal situs - describe lungs and abd viscera

-Situs Solitus - spleen, panc, stomach, sigmoid colon on L -Usual Arrangement
- Liver, cecum, appendix on R
- L lung = 2 lobes and LMSB is longer, more horizontal &
is hyparterial (inf to LPA)
- R lung = 3 lobes and RMSB is shorter, & is eparterial (post
to RPA)
-Situs Inversus - mirror image of normal -Mirror Image Arrangement
-Situs Ambiguous - symmetric arrangement, and inconsistent
- may have absent spleen, midline liver, similar bronchi and
lobe number (2 or 3) on each side
- assoc w heterotaxy syndrome = lack of lateralization of
viscera, splenic anomalies, &CHD
- some speak of "predominant situs" if most organs
follow a specific pattern
2. Cardiac Position =spacial location of most of the heart
mass
-also describe orientation of the long axis (AV jct to apex)
-Levocardia
-Mesocardia
-Dextrocardia
-Primary Dextrocardia - bc of structural CHD
-Secondary Dextrocardia - bc heart pulled/pushed to
R bc of an extracardiac anomaly (PTx, emphysema, CDH...)
-Ectopic Cordis = partly/completely exteriorized
-e.g. Pentalogy of Cantrell- defic ant diaph, defic
diaph
pericardium, CHD, defic lower sternum
3. Segment-by-Segment Cardiac Analysis (see remaining steps)
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4. Atrial Situs Anderson focuses only on the

-NOT based on venous return, but rather on their morphology appendages as the
-RA - has crista terminalis and tinea sagittalis determining factor of
- septal surface: sup and inf limbic bands of fossa ovale sidedness, and states that
- RA Appendage = broad based, triangle, more ant then LAA nearly always does this
- Pectinate muscle extends twd AV vlv and Eustach vlv correlate with the bronchial
RA Anatomy: tree sidedness.
-Sinus Venosus = IVC, SVC, CS orifice- smooth
-separated fr rest by Thebesian vlv and Esutach vlv
-RAA = broad, triangle w course trabecs
-Crista Terminalis = prominent muscle bar separating sinus venosus
fr the RAA; contains SA Nd
-Interatrial Portion of AV Canal- includes base of atrial septum & the
TV annulus
-Fossa Ovale - forms interatrial septum, w oval shaped muscular
boundary = septum 2ndum aka FO limbus
-floor of the fossa is covered w septum primum
-LA - LAA is more posterior than RAA, thinner and longer
- Pectinate muscle is only in the LAA
- septal surface has septum primum (FO valve)
LA Anatomy:
-Pulm Venous Part
-LAA - narrow and long
-AV canal region- bordered distally by MV annulus
-Septum primum attaches to LA septal surface
Situs Solitus, Inversus, or Ambiguous

Sidedness Determinants:
-RA - crista term, tinea sag, pectinate extends twd AV vlv
- appendage is broad, triangle anterior
- septum secundum (+FO limbus)
- receives major horn of the sinus venosus (IVC, SVC, CS)
-LA -pectinate muscles in appendage only
-appendage is long and narrow, more posterior
-septum primum (FO valve)
-Nly gets pulm veins
5. Ventricular Loop (situs) - must ID which is LV and which is R
-3 chambers lay bn AV canal and GAs:
-LV Anderson describes each
-RV sinus ventricle as having 3 parts
-2 parts to the Nl RV (not 2 in RV). Each has an
-RV sinus and infundibulum Inlet (AV vlv and it's
-Infundibulum (aka conus) attachements), Sinus (main
-RV - externally more triangular & its diaph wall makes acute angle cavity), and an outlet.
w ant wal
- internally:
-septal attchmts of TV (septophilic) (unlike MV which is
septophobic) Almost never ever will you
-course trabeculations have a true single
-septal surface contains moderator and septal bands ventricle. There is nearly
- infundib is incorporated into the RV sinus, so it can be hard always a remnant of the
to delineate where they separate. hypoplastic ventricle.
-but remember they come fr diff embryo structures and w xx don't
incorp well in ea other (e.g. double ch RV) or completely disassoc
(L-TGA)
-LV - externally more cone shaped
- internally:
-fine apical trabeculations
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-MV attaches via 2 distinct pap muscles, to LV free wall

(septophobic attachments)
-Ao valve is in fibrous continuity with MV bc NO conus muscle in
between the two
-bc CHD can alter this, the most reliable LV characteristic is a
smooth superior septal wall
LV Situs:
-after ID'ing ventric type, then assess the looping
-important bc helps w cor art distrib, conduction system anatomy,
etc
-L-looping --> more AV block, Ebstein like L sided TV, hypoplastic L
sided RV sinus
-Discuss in terms of chirality, because the right-left location varies
widely depending on heart position in the chest...
-ID the inflow, outflow, and septum. Put your thumb in inflow,
fingers in outflow, palm on septum of the RV. If you can do this with Anderson describes it as Right
your right hand then it is D-Looped and if you can do this with your Handed and Left Handed
left hand then it is L-Looped. (You could do the same thing with the Topology
L hand to the LV.)
6. AV Alignments and Connections

-Think of it as a continuum
-Types: Anderson stresses the
-AV Concordance = RA to RV, LA to LV difference between Alignment
-AV Discordance = RA to LV, LA to RV and Connection
-Common AV Valve = Single connection -Alignment = relation of atria
-Tri Atresia = no connection to RV to ventricle, regardless of
-Mitral Atresia = no connection to LV connection, thus you can have
-Overriding TV = RA over both ventricles (though not always AV concordance even with Tri
RA) Atresia
-Overriding MV = LA to both ventricles (though not always LA) -Connection = opening bn the
-Straddling TV two chambers
-Straddling MV
-Double Inlet Ventricle = both AV valves to same ventricle
-Straddling = the AV vlv has some attchmts to either side of IVS
-Overriding = all the AV vlv attchmts go to same (ipsilat) side of IVS
7. VA Alignments
-From which ventricle does each GA originate
-Describes how the semilunar valves and their vessels align
with the ventricle (not with each other, and not based on if
there's an infundib)
-note though that there is a continuum...
-easy to describe if the GA is near completely fr one ventricle,
hard to say if it overrides the IVS much; the 50% rule is no
good, so in this case best to describe how it overrides
8. Type of Infundibulum (conus)
= the connecting part bn the ventric and the GA
-Nl'y there's a complete subpulm conus that separates pulm &
AV vlv
-w subAo conus it's incomplete--> AV-Ao vlv continuity
-the part that forms the "conus septum" bn the ant-lat side of
the LVOT (under the R cor cusp) and the RVOT
-4 options
-SubPulm (without subAo free wall infundib) = Nl
-SubAo (without subPulm free wall infundib) = in TGA
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-Bilateral Conus = in DORV, rarely in TGA

-Bilateral Absent Conus = in some pts w DOLV
9. Semilunar Valve Relationship

-Describes relationship at the level of the valve
-Note that in the Nl heart the Ao vlv is to the RIGHT &
POSTERIOR
of the PA vlv bc the conus is there they cross over each
other...
-must consider both the D & L relationship, and the A & P
relationship.
-Solitus = Ao R/P to PA
-Inversus = Ao L/P to PA
-D = Ao R/A to PA
-L = Ao L/A to PA
-A = Ao directly Ant to PA
10. Associated Anomalies

-either by hemodynamic importance or anatomic order
-e.g. anomalous systemic/pulm venous return, septal
defects, etc
Terminology
Van Praagh:
-decide sidedness separately for atria, ventricles and GAs
-Atria- S Solitus, I Inversus, A Ambiguous
-Ventricles- D D-looped (Solitus), L L-looped (Inversus), X
Ambiguous
-Great Arteries- S Solitus, I Inversus, D D-transposed/malposed, L
L-transposed/malposed, or A Ambiguous or Anterior
transposition/malposition
-Then use parentheses (e.g. TGA is SDD, normal is SDS)
Sequential Segmental Analysis

-go by the flow -start with systemic veins all the way through the GAs
Cardiac Position
-Location in chest - Dextroposition/Mesoposition/Levoposition
-Heart Orientation (apex direction)-
Dextrocardia/Mesocardia/Levocardia
-apex location doesn't necessarily correlate to heart sidedness,
though midline apex is assoc w situs ambiguous
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-Pulmonary Sidedness Bilateral right-sidedness and

-RPA is anterior to the upper lobe bronchus bilateral left sidedness are
helpful mnemonics for
-LPA goes posterior to the upper lobe bronchus teaching, but not as basic
-Upper lobe bronchus is longer than right (1.5-2x, regardless of biology. For example, the RA
Ao arch sidedness) is not really bilateral in
-Eparterial = bronchus above PA (R bronchus), and Hiparterial = asplenia, nor is the LA really
bilateral in polysplenia
bronchus under PA (L bronchus)
Abdominal Sidedness
-liver right, stomach left
-Asplenic pts (R isomerism)- usually midline liver, single gallbladder;
often w GI malrotation
-stomach/panc can be anywhere
-Aorta & IVC on same side of vertebrae
-Polysplenia (L isomerism)- spleens on same side of vertebrae
-often interrupted Ao arch
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Morphology of Cardiac Segments

-note that Single = contralateral structure is absent, Common =
comm'n bn the 2 sides...
SVC- +/- LSVC
IVC
CS
-usually drains to RA, rarely is the os atretic w a LA fistula
-partial unroofing can happen
-complete unroofing = absent CS, assoc w asplenia...
-CS and Ligament of Marshall remnant of L cardinal vn
Pulmonary Veins
-w LA isomerism, may have 2 to each side
-having only 3 vns may be Nl variant (usually bc the L vns merge prior
to LA connection)
-w APVR, may go to a vertical vn/ascending vn to L
brachiocephalic/innominate vn
-the vn may be aka persistent SVC, vertical vn, levo-atrial cardinal
vn
Atria
-RA...
-LA... - only the foramen and the LAA is derived fr embryologic LA,
remainder is fr PVns..
-Common Atrium- near absent atrial septum..., +/- AVC defect
AV Valves
-TV annulus attaches more apical than MV annulus
-TV
-septophilic leaflets
-3 leaflets, 3 commissures, 3 paps elliptical annulus but triangle
shaped orifice at level of the cords
-ant leaflet of TV separates inflow and outflow in RV, along with the
infundibulum...
-MV
-mitral Ao continuity in Nl heart (Ant leaflet of MV helps form part of
LVOT)
-2 leaflets, 2 paps
-septophobic
-Common AV Valve
-R and L AV vlvs... (after septation, don't refer to them as TV/MV...)
Ventricles
-rarely does one have a truly single ventricle...
-RV- thicker trabeculations
-LV- finer trabeculations
-Common Ventricle
-Hypoplastic Ventricle...
Semilunar Valves
-
Ao
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Arch Sidedness
-R Arch has mirror image branching relative to a Nl L arch...
-PAs
-PA large Y shape vs bronchial arts come fr desc Ao and course along
the bronchi...
-collaterals look similar to the bronchial arts...
Truncus Arteriosus = Common Truncal Artery

...
Ductus Arteriosus = Ductal Artery
...
Positions of Cardiac Segments
1. Cardiac Position:
• Levocardia: left-sided within the thorax
• Dextrocardia: right-sided within the thorax
• Mesocardia: centrally located within the thorax,
• Ectopia cordis: partially or completely outside the thorax
2. Situs :
• Situs Solitus: normal arrangement of viscera and atria, with RA
right-sided and LA left-sided.
• Situs Inversus: inverted arrangement of viscera and atria, with RA
left-sided and LA right-sided, as in a mirror-image of situs solitus.
• Situs Ambiguus: the ambiguus situs often associated with congenital
asplenia, less often with polysplenia, and rarely with a normally
formed spleen. In situs ambiguus, the basic type of visceroatrial
situs is anatomically uncertain or indeterminate, because the
anatomic findings are ambiguous. Situs ambiguus can be down into
two types of situs:
• Asplenia: bilateral right-sidedness,
• and Polysplenia: bilateral left-sidedness. Hence, we prefer: Situs
ambiguus with asplenia, and Situs ambiguus with polysplenia.
Heterotaxy Syndrome: situs ambiguus.
3. Atrio- ventricular concordance (looping):

Loop simply says where the RV inflow tract is. Note that the RV is the
right ventricular inflow tract (sinus, or body). The outflow tract is the
infundibulum or conus (very different from the true RV anatomically,
embryologically and positionally).
• D-loop: RV right-sided relative to LV. Synonyms for D-loop:
normally located ventricles, solitus ventricles, noninverted ventricles
in situs solitus.
• L-loop: RV left-sided (levo or L) relative to LV.
• In situs ambiguus, D-loop or L-loop ventricles cannot be described
as noninverted or inverted, because the frame of reference, the type
of visceroatrial situs, itself is unknown.
• X-loop: type of bulboventricular loop is anatomically uncertain or
unknown.
• Concordant Loop: the ventricular loop is concordant or appropriate
relative to the visceroatrial situs.
In situs solitus, a D-loop; in situs inversus, an L-loop; and in situs
5
14
15

ambiguus, the concordance / discordance concept is not applicable.

• Discordant Loop: ventricular loop is discordant or inappropriate
relative to the visceroatrial situs.
In situs solitus, an L-loop; in situs inversus, a D-loop; and in situs
ambiguus, the concept is inapplicable.
• Mixed: discordant; e.g., mixed levocardia with atrial noninversion
means left-sided heart with situs solitus of viscera and atria and
discordant or inappropriate ventricles. Since the atria are in situs
solitus, the ventricles are inverted (L-loop).
• Ventriculo-arterial concordance
• It is the usual normal relationship between the great arteries
themselves and between the great arteries and the ventricles. An
aortic valve arising above LV is to the right of the pulmonary valve
which is arising above RV and the pulmonary artery (PA) always
passes to the left of the ascending aorta.
• Ventriculo-arterial discordance
• Transposition of the Great Arteries (TGA): Aorta arising above RV
and PA arising above LV.
• D-TGA: aortic valve to right (dextro or D) relative to pulmonary
valve.
• L-TGA: aortic valve to left (levo or L) relative to pulmonary valve
• A-TGA: aortic valve directly anterior (antero or A) relative to
pulmonary valve.
• Complete TGA: physiologically uncorrected TGA; e.g., in situs
solitus, usually D-TGA, and in situs inversus, usually L-TGA.
• Corrected TGA: physiologically corrected TGA; e.g., in situs solitus,
usually L-TGA, and in situs inversus, usually D-TGA.
• Malposition of the Great Arteries (MGA): the great arteries are

malposed, but they are not transposed. In TGA, both the Ao and the
PA are placed across the ventricular septum and so arise above the
anatomically inappropriate ventricles. In malposition of the great
arteries (MGA), this is not the case. MGA occurs, for example, with
DORV and DOLV.
• Double Outlet Right Ventricle (DORV): Origin of Ao and PA entirely
or predominantly above RV.
• Double Outlet Left Ventricle (DOLV): origin of Ao and PA entirely or
predominantly above LV.
• D-MGA, L-MGA and A-MGA: MGA with the AoV to the right (dextro
or D), to the left (levo or L), or anterior (antero or A) relative to the
PV, respectively.
• Single (Common) Ventricle: MGA applies accurately, but TGA does

not.
Ø (type A) single ventricle: is the most frequent form of single
ventricle, single LV with infundibular outlet chamber (double inlet
LV). The malformed ventricular septum is present which is well
developed on the LV side but virtually absent on the RV side
(because the RV is absent).
Ø (type B) single RV: when there is no identifiable ventricular
septum
Ø (type C) single ventricle: absent or rudimentary ventricular septum
Ø (type D)single ventricle: morphologically unidentified ventricular
myocardium
15
16

Fetal circulation
General Pathologic Considerations
Classification of congenital heart disease

Algorithm for the classification of CHDs (5 questions and 6
Groups).
16
17

Blue, shocky infant Gray baby Blue, well-perfused Pink, shocky infant
baby
Shock - Not enough Happy Cyanosis - not Sick Cyanosis - Happy Tachypnea -
systemic flow enough PBF pulmonary venous too much PBF
congestion
-Critical AS -Severe PS (Critical -Obstructed TAPVR Acyanotic
-Ao Arch hypoplasia/ PS) -TGA/IAS/restrictive -Moderate/Large VSD
-Co/A -PA/IVS atrial septum -Large PDA
-AVSD
-IAA -PA/VSD (aka severe -HLHS/IAS/restrictive
-PTA
-HLHS TOF) atrial septum
-APW
-TOF -Unobstructed TAPVR
-Tricuspid Atresia (if Cyanotic
w restrictive VSD or -PTA
signif PS) -DORV/VSD no PS
-TGA/VSD without a
restrictive atrial septum
-FSV with no outflow
tract obstruction
FIGURE 1−9. Algorithm for the classification of CHDs (5 questions, 4 sick infants, and 6
pediatric groups).
17
18

Clinical Picture & Diagnostic Considerations
Diagnosing CHD in the Sick Infant

Categories of an infant with CHD Categories of an infant with
PGE likely will help: CHD
4. Shock - not enough Qs PGE likely will help:
5. Happy Cyanosis - not enough Qp • Shock - not enough Qs
PGE likely won't help: • Happy Cyanosis - not
• Sick Cyanosis - = pulmonary congestion, pulmonary blood enough Qp
flow cannot get out PGE likely won't help:
• Happy Tachypnea - pulmonary overcirculation • Sick Cyanosis - =
pulmonary congestion,
pulmonary blood flow
cannot get out
• Happy Tachypnea -
pulmonary overcirculation
Shock - Not enough systemic flow

Blue, shocky infant
Blue, shocky infant

-bc of obstruction to Qs; the PDA no longer compensates for the
problem (bc it is closing)
-Critical Aortic Stenosis
-Ao Arch hypoplasia/Coarctation
-Interrupted Aortic Arch
-Hypoplastic Left Heart Syndrome
-Sx
-tachycardic
-lethargic
-grey/pale/mottled
-no murmur usually (sometimes + w AS)
-cool limbs
-Low BP
-All are ductal dependent for Qs = need the PDA to supply Q to lower
body. In some, need PDA to supply the coronaries & head/neck vessels
via retrograde Q thru the arch (e.g. AS, HLHS)
--> lower extrem SaO2 will be low (bc get's Q fr the PA via the PDA),
and if AS & HLHS, the upper extrem will have low SaO2 too (bc the
blockage is prior to the head/arm vessels so they get retrograde Q via
the PDA)
-Often have Differential Cyanosis = upper extremities have higher
SaO2 than lower
-CXR - varied, usually Nl w AS & CoAo; IAA often assoc w VSD so --
> overcirc; HLHS--> overcirc
Happy Cyanosis - not enough pulmonary flow

Gray baby
18
19

Gray baby
-bc of obstruction to Qp
-Ductal dependent
-if PDA open--> mild/mod cyanosis, much worse as it closes
-Severe PS (Critical PS)
-Pulm Atresia w Intact Ventricular Septum
-Pulm Atresia/VSD (aka severe TOF)
-Tetralogy of Fallot
-Tricuspid Atresia (if w restrictive VSDor signif PS)
-Sx
-cyanosis
-mild tachypnea, d/o degree of hypoxia
-+/- murmur (PS yes, pulm atresia no)
-CXR- Nl to dark lung fields
Sick Cyanosis - pulmonary venous congestion

Blue, well-perfused baby
Blue, well-perfused baby

-bc of pulm vn return obstruction--> can't exit--> signif resp
distress, cyanosis, low CO
-*** Likely one of these with really sick newborn at <24 hrs of life
-Obstructed TAPVR
-TGA with intact/restrictive atrial septum
-HLHS with intact/restrictive atrial septum
-Sx
-Hypoxia
-resp distress- tachypnea/retractions
-+/- poor output w cool extrem/pallor
-Get sick quickly
-CXR- pulm edema
Happy Tachypnea - too much pulmonary flow

Pink, shocky infant
Pink, shocky infant

-bc of pulmonary overcirculation (Qp:Qs usually >2:1), but cardiac output
is maintained
-includes L to R shunts...
-Moderate/Large VSD
-Large PDA
-AV Canal Defect
-Truncus Arteriosus
-Aortopulmonary Window
-Unobstructed TAPVR
-Sx
-tachypnea
-retractions- but usually comfortable
-cardiomegaly, hepatomegaly
-poor PO, poor wt gain
-if cyanotic- the pt must also have a mixing lesion:
-Truncus Arteriosus
-DORV with no PS
-TGA with VSD without a restrictive atrial septum
-Single ventricle with no outflow tract obstruction
10
19
20

-CXR: increased pulmonary vascularity
What to check before the echo?

-CXR • CXR
-4 extrem BP - r/o CoAo • 4 extrem BP - r/o CoAo
-Pre & Post SaO2 (both upper and at least 1 lower) • Pre & Post SaO2 (both upper
-ECG (?sensitivity for a specific lesion) and at least 1 lower)
• ECG (? sensitivity for a
specific lesion)
CXR:
-Shock- Nl/increased pulm vasc, cardiomegaly
-if Left sided obstructive lesion- may cause incr Qp if single
ventricle or if VSD
-Happy cyanotic- Nl/decr pulm vasc; Nl cardiac size
-often Nl when PDA open, then decr when it closes (looks like
a TOF CXR)
-Sick Cyanotic- pulm edema, cardiomegaly
-Happy Tachypnea- incr pulm vasc, +cardiomegaly
4 Extremity BP
-DDx CoAo from other L sided obstructive lesions
-SBP in R arm should be at least 10mmHg higher than SBP in legs if
+CoAo
-Check both upper extrem bc pt might have an aberrant R
subclavian that comes fr descending Ao, after the PDA insertion (site of
coarctation), so will be low/Nl and similar to that of leg, giving a false
negative for coarctation
-in HLHS & AS they are NOT abnormal
-if IAA, can be misleading if the PDA is open bc it still gets good
Q...
Pre & Post Ductal O2 Sats

-No diff:
-R sided lesions (PS/Pulm Atresia)
-TAPVR
-Truncus
-Isolated intracardiac shunts (VSD) and AP Windows
-HLHS w Aortic Atresia (no oxygenated blood going forward to
create a difference)
-Pre>Post = PDA is open w a mainly R to L shunt to the lower body,
w arch supplied by the Ao
-Pulm htn (>/= systemic P)
-L heart obstructive lesions - critical AS, Interrupted Ao Arch,
Coarctation of the Aorta
-Post>Pre = Reverse Differential Cyanosis = TGA + PDA + one of
the following:
-TGA + Coarctation of the Aorta or IAA
-TGA + Pulmonary Hypertension
Hyperoxia Test
= apply 100% FiO2 and check PaO2: Fail = PaO2 is <100-
250mmHg on 100%FiO2 for 10 minutes; can only be done by oxyhood
or ET tube.
-helps DDx bn an O2 responsive (noncardiac) etiology of hypoxia,
vs a cyanotic CHD
-Not as useful if the echo machine is available
-Everyone discussed above will fail the test, except for happy
11
20
21

cyanotics with no mixing lesions, critical left sided lesions if the duct is
closed (but then they'd be dead)
Mgt
-Shock - L sided obstruction lesions

-PGE - bc all are ductal dependent
-Improve the acidosis - sedate, ETT, pressors...
-When stable--> correct/palliate
-AS: Balloon valvuloplasty
-CoAo/IAA: repair the arch
-HLHS: Norwood/BTS
-Happy Cyanotic - too little Qp

-PGE - bc often ductal dependent
-O2
-Supply a Qp source:
-BT shunt, PDA stent, Pulmonary Balloon valvuloplasty, complete
repair (TOF)
-Sick Cyanotics - pulmonary congestion

-STAT cath lab for intact/restrictive atrial septum (HLHS, TGA, AS,
etc)
-STAT operation - obstructive TAPVR
-PGE if HLHS or TGA
-check the atrial septum early:
-intact/restrictive?
-R to L shunting? (TAPVR)
-Happy Tachypnics - pulmonary overcirculation

-less emergent
-usually don't need PGE (already have much Qp, no problem w Qs)
-diuretics, possible afterload reduction to promote Qs rather than
Qp
-Will need surgical repair (often at 3-6mo or late, varies by
Sx/lesion)
-if CHF Sx despite Rx, c/s a PA band
-except for truncus & AP windows- overcirculation in these
pts can be severe, and result in NEC, so repair as a a newborn
12
21
22

FIGURE 1−2. The 3 cardiac segments, the 5 steps tp detect abnoralities of cardiac loop.
13
22
23

FIGURE 1−4. Algorithm for systematic ECHO exam.
14
23
24

Systematic ECHO exam

M-Mode
- = a single pt (ice-pick view) over time
-transducer @ LSB
-√chamber/vssl dimensions, wall/septal thickness
-√LV sys fx
-√valve motion- MVP, MS, phtn, & IV septal
motion
-√pericardial fluid
-Normals
-√ during diastole, w QRS onset,e xcept for LA &
LV dimensions- check during systole.
-LV sys fx
-Fractional Shortening- FS% = Dd-
(Ds/Dd)*100
= degree the muscle shortens systole. ~to fx if no
A cross-sectional view of the left side of the
regional wall motion defect & LV contractility is heart along the long axis (top) through
concentric; D=dimension, d=diast, s=sys. Avg = which “ice-pick” views of the M-mode echo
recordings are made (bottom). Many other
35% (28-44 range). w LV that is poorly M-mode views are possible, but only three
compensated for P overld, V overld, myocardial are shown in this figure. The dimension of
d/o, doxorubicin xx; but in vol overld (VSD, the aorta (AO) and left atrium (LA) is
measured along line (1). Systolic time
PDA, AR, MR) & P overld (AS, HOCM) intervals for the left side are also measured
-EF%= Dd3 –(Ds3/Dd3)x100; this makes at the level of the aortic valve (AV). Line (2)
assumption that minor axis of LV = ½ major LV passes through the mitral valve.
Measurements made at this level are not
axis, but in kids this is wrong!. Avg = 66% (56- very useful in pediatric patients.
78%) Measurement of chamber dimensions and
wall thickness of right and left ventricles is
-Systolic Time Intervals- =preejection pd + made along line (3). The following
ventricular ejection time;
-PEP- preejection pd = time from Q wave till
Doppler
semilunar valve opening;
-reps rate of P in ventric during sys during
Pressure Gradiants
isovolumetric systole (dp/dt)
-Bernoulli equation- P1-P2
-VET- ventric eject time = cusp opening of
(mmHg) = 4(V22 – V12)
semilunar valve to cusp closing
………
-both are affected by HR, but their ratio stays
………
same despite change in HR
………
-LV failà LPEP & LVETà LPEP/LVET ratio. =
more time to P, less ejection time
-ASà LPEP & LVETà LPEP/LVET ratio = P
quicker, more ejection time
-Nl LPEP/LVET ratio = 0.35 (0.30-0.39)
Two-Dimensional Echos
-directs a plane (not a spike like M mode)
-Prasternal –
-Long Axis
-Standard View: TOF VSD, pericardial
effusion, MVP,
-RV inflow view: TR, RV inflow region, VSD at
AV valve & trabecular VSD, & RA appendage
15
24
25

-RV outflow view: RVOT, pulm vlv, prox main

PA
-Short Axis
-Ao View: AoValve at center w RVOT ant to it
& main PA R of it (circle & sausage view). Good
for Ao vlv cusps, PR/PS,
-Coronary Arteries-
-Mitral Valve- see fish mouth
-Papillary muscles
-Apical
-Four Chamber View – see Coronary sinus
view, apical 4 chamber view, 5 ch view
-Apical Long Axis View -
-Subcostal
-Suprasternal notch
Diagram of a family of parasternal short-axis views.

Semilunar valves and great artery level (A), coronary
arteries (B), mitral valve level (C), and papillary muscle
level (D). AO, aorta; LA, 1eft atrium; LCA, left coronary
artery; LPA, left pulmonary artery; LV, left ventricle;
MPA, main pulmonary artery; MV, mitral valve; PM,
papillary muscle; RA, right atrium; RCA, right coronary
artery; RPA, right pulmonary artery; RV, right ventricle;
RVOT, right ventricular outflow tract.
Diagram of important two-dimensional echo views

obtained from the, parasternal long-axis transducer
position. Standard long-axis view (A), RV inflow view
(B), and RV outflow view (C). AO, aorta; CS, coronary
sinus; Desc. Ao, descending aorta; LA, left atrium; LV,
left ventricle; PA, pulmonary artery; RA, right atrium;
RAA, right atrial appendage; RV, right ventricle.
16
25
26

Apical long-axis views. A, Apical three-chamber view.

B, Apical two-chamber view. AO, aorta; LA, left atrium;
LAA, left atrial appendage; LV, left ventricle; RV right
ventricle.
Diagram of two-dimensional echo views obtained with

the transducer at the apical position. A, A posterior
plane view showing the coronary sinus. B, The
standard apical four-chamber view. C, The apical “five-
chamber” view is obtained with further anterior
angulation of the transducer. AO, aorta; CS, coronary
sinus; LA, left atrium; LV, left ventricle; RA, right
atrium; RV right ventricle.
Subcostal short-axis (sagittal) views. A, Entry of venae

cavae with drainage of the azygos vein. B, View
showing the RV, RVOT, and pulmonary artery. C,
Short-axis view of the ventricles. Azg. V, azygos vein;
LA, left atrium; LV, left ventricle; MV, mitral valve; PA,
pulmonary artery; RA, right atrium; RPA, right
pulmonary vein; RV right ventricle, SVC, superior vena
cava, TV, tricuspid valve.
17
26
27

Diagram of subcostal long-axis views. A, Coronary

sinus view posteriorly. B, Standard subcostal four-
chamber view. C, View showing the left ventricular
outflow tract and the proximal aorta. D, View showing
the right ventricular outflow tract (RVOT) and the
proximal main pulmonary artery. AO, aorta; CS,
coronary sinus; LA, left atrium; LV left ventricle; PA,
pulmonary artery; RA, right atrium; RV right ventricle,
SVS, superior vena cava.
Abdominal views. Left, abdominal short-axis view.

Right, abdominal long-axis views. AO, aorta; CA, celiac
axis; HV, hepatic vein; IVC, inferior vena cava; RA,
right atrium; SMA, superior mesenteric artery.
18
27
28

Diagram of suprasternal notch two-dimensional echo

views. Top, Long-axis view. Bottom, Short-axis view.
AO, aorta; Asc. Ao, ascending aorta; Desc. Ao,
descending aorta; Inn. A, innominate artery; Inn. V,
innominate vein; LA, left atrium; LCA, left carotid
artery; LSA, left subclavian artery; MPA, main
pulmonary artery; PA, pulmonary artery; RPA, right
pulmonary artery; SVC, superior vena cava.
Diagram of subclavicular views. A, Right

subclavicular view. B, left subclavicular view. AO,
aorta; IVC, inferior vena cava; LPA, left pulmonary
artery; MPA, main pulmonary artery; RA, right
atrium; RPA, right pulmonary artery; RV, right
ventricle; SVC, superior vena cava.
Treatment Considerations
Mgt
-Shock - L sided obstruction lesions

-PGE - bc all are ductal dependent
-Improve the acidosis - sedate, ETT, pressors...
-When stable--> correct/palliate
19
28
29

-AS: Balloon valvuloplasty

-CoAo/IAA: repair the arch
-HLHS: Norwood/BTS
-Happy Cyanotic - too little Qp

-PGE - bc often ductal dependent
-O2
-Supply a Qp source:
-BT shunt, PDA stent, Pulmonary Balloon valvuloplasty, complete
repair (TOF)
-Sick Cyanotics - pulmonary congestion

-STAT cath lab for intact/restrictive atrial septum (HLHS, TGA, AS,
etc)
-STAT operation - obstructive TAPVR
-PGE if HLHS or TGA
-check the atrial septum early:
-intact/restrictive?
-R to L shunting? (TAPVR)
-Happy Tachypnics - pulmonary overcirculation

-less emergent
-usually don't need PGE (already have much Qp, no problem w Qs)
-diuretics, possible afterload reduction to promote Qs rather than
Qp
-Will need surgical repair (often at 3-6mo or late, varies by
Sx/lesion)
-if CHF Sx despite Rx, c/s a PA band
-except for truncus & AP windows- overcirculation in these
pts can be severe, and result in NEC, so repair as a a newborn
20
29
30

Jahangiri M, Lincoln C,
Palliative procedures for CHDs Shinebourne EA. Does the modified
Blalock-Taussig shunt cause growth of
A palliative operation does not correct but is required to improve an the contralateral pulmonary artery?
abnormal heart function, minimizing the disorder, usually in children Ann Thorac Surg. 1999
too young for corrective surgery. The aim is to lessen cyanosis, May;67(5):1397-9.
control heart failure or prepare the circulation for later correction Godart F, Qureshi SA, Simha A,
when the baby grows up to an age and body weight that are suitable Deverall PB, Anderson DR, Baker EJ,
for the available techniques. Tynan M. Effects of modified and
There are 21 palliative procedures in total, which have been classic Blalock-Taussig shunts on the
categorized into four classes, according to their goals and indications: pulmonary arterial tree. Ann Thorac
• éPBF for pulmonary oligaemia (including shunt procedures); Surg. 1998 Aug;66(2):512-7;
• ê PBF for pulmonary overcirculation (pulmonary banding and discussion 518.
Norwood procedure); Gladman G, McCrindle BW,
• é intracardiac blood-oxygen mixture for systemic hypoxaemia Williams WG, Freedom RM, Benson
(atrial septostomy subjected to different techniques); LN. The modified Blalock-Taussig
shunt: clinical impact and morbidity in
• other procedures, including congenital mitral or aortic stenosis Fallot's tetralogy in the current era. J
palliation, coarctation of aorta palliation and hybrid palliative Thorac Cardiovasc Surg. 1997
procedures for hypoplastic left heart syndrome (HLHS). Jul;114(1):25-30.
A) Shunt procedures Odim J, Portzky M, Zurakowski
• A systemic-to-pulmonary artery shunt has been used for many D, Wernovsky G, Burke RP, Mayer JE
years to: Jr, Castaneda AR, Jonas RA.
Ø establish unobstructed systemic blood flow, Sternotomy approach for the modified
Ø normalize PBF and pressure Blalock-Taussig shunt. Circulation
Ø relieve PVO. 1995 Nov 1;92(9 Suppl):II256-61.
• An ideal shunt is expected to have the following attributes:
Ø technical simplicity,
Ø good functionality,
Ø good long-term patency,
Ø easy takedown before repair and no residual shunt after
closure.
• The indications for a systemic-to-pulmonary artery shunt
include
Ø TOF,
Ø TA,
Ø PA.IVS, PA/VSD
Ø Ebstein’s anomaly,
Ø FSV situation with pulmonary or aortic atresia and HLHS.
• There are 11 shunt procedures in total.
1. Blalock-Taussig’s shunt (BTS) Ullom RL, Sade RM, Crawford
• The classic BTS is a direct anastomosis between the FA Jr, Ross BA, Spinale F. The
transected subclavian artery (or the innominate artery) and Blalock-Taussig shunt in infants:
the pulmonary artery. It does not require the use of prosthetic standard vs. modified. Ann Thorac
material and offers the theoretical possibility for growth Surg 1987 44(5):539-43.
• The major disadvantages of classic shunts were: Arciniegas E, Farooki ZQ, Hakimi
Ø requires extensive surgical dissection M, Perry BL, Green EW. Classic
shunting operations for congenital
Ø long operative dissection time,
cyanotic heart disease. J Thorac
Ø phrenic nerve injury,
Cardiovasc Surg 1982 84(1):88-96.
Ø technical difficulties during takedown and
Ø sacrifices the subclavian artery and possible arm ischaemia
• After a classic shunt, distortion is usually explained by the
failure of the anastomosis to grow and the effects of the scar
tissue around the suture material.
• When classic shunts are performed, it is usually reported that
they should be constructed on the side opposite to the aortic
arch to avoid pulmonary artery kinking
21
30
31

• Better understanding of the condition led to a revolution in the

surgical technique for the Blalock-Taussig’s shunt: an
interposed graft (prosthetic or human vascular) was used
between the subclavian and pulmonary arteries ; this is called
a modified Blalock-Taussig’s shunt (MBTS) which avoided
many of the disadvantages of the classic shunt
• The commonest complications following MBTS include shunt
thrombosis leading to obstruction, shunt stenosis, infection,
peri-shunt (graft) seroma, pseudoaneurysm and pulmonary
artery distortion.
2. Central shunt
• A central shunt is an anastomosis between the ascending
aorta and the main pulmonary artery, made of prosthetic or
other materials; it is also known as the Mee’s shunt .
• The internal mammary artery is used to create a systemic-to-
pulmonary artery shunt after failure of a previous BTS. This
offers the advantage of growth and flow adaptation, Eliminates
the risk of prosthetic graft infection, and doesn’t have arm
ischemia effect.
• Advantages of central shunt:
Ø its applicability in small children with small peripheral vessels,
Ø prevention of distortion of pulmonary arteries,
Ø provision of equal pulmonary blood flow to both lungs,
Ø lower occlusion rate,
Ø avoidance of subclavian artery steal
Ø ease of closure during corrective repair.
• The primary disadvantages are entry into the pericardium and
inapplicability in patients without a PDA or other source of
PBF.
3. Glenn’s shunt )BDGM CPA, CPS, PCPA Murthy KS, Coelho R, Naik SK,
• A connection between the SVC and the RPA (end to end Punnoose A, Thomas W, Cherian KM.
anastomosis) is known as a classic Glenn’s shunt Novel techniques of bidirectional
• It has been performed to improve PBF in patients with diverse Glenn shunt without cardiopulmonary
cyanotic CHD. bypass. Ann Thorac Surg. 1999
• The Glenn’s shunt does not create volume overload of the ventricle Jun;67(6):1771-4.
or increased work for the ventricle, as is the case with systemic- McElhinney DB, Marianeschi
SM, Reddy VM. Additional
pulmonary artery shunts. It provides venous flow to the lung fields
pulmonary blood flow with the
for oxygenation, rather than an arteriovenous mixture.
bidirectional Glenn anastomosis: does
• The BDG is performed by connecting the SVC to the RPA (end to it make a difference? Ann Thorac
side anastomosis) using fine sutures and by dividing or tying up the Surg. 1998 Aug;66(2):668-72.
PA. Kostelka M, Hucin B, Tlaskal T,
• It decreases volume load on the single ventricle while improving Chaloupecky V, Reich O, Janousek J,
oxygen saturation Marek J, Skovranek J. Bidirectional
• The BDG is preferred in very small babies, especially those aged Glenn followed by total
less than 2 years. The BDG is equivalent physiologically to half a cavopulmonary connection or primary
Fontan’s shunt, hence it is also referred to as a hemi-Fontan total cavopulmonary connection? Eur J
procedure. Cardiothorac Surg. 1997
• Progressive cyanosis, which may be due to the development of Aug;12(2):177-83.
systemic venous collaterals that decompress the superior caval
system into the inferior caval system, and the formation of a
diffuse arteriovenous shunt not amenable to coil embolization are
the two problems that occur most frequently after a BDG.
4. Sano’s shunt
• The most recent and intriguing modification to the Norwood
procedure was the use of the RV to PA conduit, referred to as the
Sano modification of the Norwood procedure,
• It allows blood to be pumped directly to the lungs. The technique
22
31
32

was adapted after its initial description and greater haemodynamic

stability was noted with this Sano modification than with the MBTS.
5. ‘‘Wanna-be’’ Blalock-Taussig’s shunt
• Ductus stenting in neonates and infants with duct-dependent
cyanotic CHD has eliminated the need for palliative surgery.
The procedure carries no risk of serious complications or pulmonary
artery distortion and stenosis and gains time for the child and the
pulmonary arteries to grow, while leaving the operative field for
definitive surgery untouched .
6. Other shunts
6.1. The Potts’s (Potts-Smith-Gibon)
• It is a connection between the descending aorta and the LPA which
was proposed initially as an alternative to the classic BTS in
neonates.
• This shunt was abandoned because of the high incidence of
subsequent pulmonary hypertension, the preferential blood flow to
one lung with kinking and distortion of the PA and the technical
difficulties with takedown.
6.2. The Waterston-Cooley’s shunt
• It is a side-to-side anastomosis between the ascending aorta and
the RPA (extrapericardial [Waterston] and intrapericardial
[Cooley]), which is indicated for TOF.
• It was largely abandoned because of its complications (including
preferential distribution of most or all shunt flow to the right lung,
narrowing or obstruction of the RPA at the anastomotic site,
increasing stenosis or atresia of the RVOT, hypoplasia of the LPA,
pulmonary overcirculation, LV failure, technical difficulty to close
this shunt properly at the time of total correction and obstruction of
the shunt itself ) and a similar mortality rate to that for the BTS,
6.3. The Shumacker-Mandelbaum’s shunt
• It was a shunt technique between the ascending aorta and either
the RPA or LPA or the MPA. A graft composed of homologous aorta
with a Dacron cuff at either end for end-to-side anastomoses was
used.
6.4. The Redo-Ecker’s shunt
• It was named after Redo and Ecker, who used a woven teflon
prosthesis anastomosed end to side between the aorta and the
MPA, just distal to the origins of these vessels within the
pericardium. In this way, the blood is delivered directly into the
proximal portion of the PA and the artery is more suitable for
enlargement, which benefits total correction in the future.
6.5. An aorta-to-right ventricle shunt was applied in only
one of 19 patients requiring a systemic-to-pulmonary
shunt, as reported by Nanton et al.
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Pinho P, Von Oppell UO, Brink

Pulmonary artery banding & the Norwood procedure J, Hewitson J. Pulmonary artery
banding: adequacy and long-term
• Pulmonary artery banding (PAB) outcome. Eur J Cardiothorac Surg
Ø PAB is the preferred method of palliation in children born with 1997 Jan;11(1):105-11.
cardiac defects characterized by LèRS and pulmonary Horowitz MD, Culpepper WS
overcirculation. 3d, Williams LC3d, Sundgaard-Riise
Ø This technique has been broadened to treat congestive heart K, Ochsner JL. Pulmonary artery
failure caused by large VSD, and AVSD. However, current banding: analysis of a 25-year
indications are: experience. Ann Thorac Surg 1989
1. VSD 48(3):444-50.
ü Multiple VSDs in early infancy
ü Coarctation of the aorta with a large VSD
ü VSD complicated by other congenital non-cardiac anomalies
(e.g. large omphalocoele, oesophageal atresia, diaphragmatic
hernia, premature baby with low birth weight, or VSD in
severely undernourished babies and the presence of severe
infection (sepsis, respiratory, skin) that cannot be brought
under control because of severe heart failure)
2. TGA
ü for rapid ventricular retraining with or without construction of
a systemic–pulmonary artery shunt
ü Severe RV failure after a Mustard or Senning operation. The
pulmonary artery is banded; unless the LV pressure is already
increased (such as in associated LVOTO). The ASO is then
performed about 12 months later, when the hypertrophied
subpulmonary LV is considered adequate to sustain the
systemic afterload
3. DILV and TA w/o PS.
4. HLHS
ü Bilateral PAB, preceded by endovascular stenting of the PDA,
is being very selectively applied as part of the Norwood
strategy in the management of HLH
Ø The degree of PAB depends on the underlying cardiac lesions.
Acute PAB may lead rapidly to ventricular failure. A more
gradual approach to PAB may be required to achieve a
successful outcome.
Ø The goal of PAB is to produce a distal pulmonary artery
pressure of 30 to 50% of systemic pressure. As the PA is
being constricted, the systemic pressure starts rising while the
distal PAP is falling. The optimal degree of constriction is
achieved when the systemic pressure reaches a plateau,
usually an increase of 10–20mmHg without bradycardia and
oxygen desaturation beyond the low 90s. Trusler and Mustard
(1972) calculated the optimal length of the band from the
infant’s body weight. If the banding is done for biventricular
heart, 20mm + weight in kg is used. If done for complex
cardiac malformations, it is 24mm + weight in kg
Ø PAB is contraindicated in the presence of significant subaortic
obstruction, such as FSV defects in which the aorta arises
from an outflow chamber, and in patients who are at high risk
of such an obstruction; in addition, it is not used in patients
with PTA.
Ø A variety of banding material is available but umbilical tape is
broad enough to minimize the risk of eroding through the
pulmonary artery wall. An adjustable PAB has been applied
since 1972. A percutaneously-adjustable band with a fluid-
filled reservoir that allows variable constriction was developed
in 1986, followed by an implantable, telemetrically-controlled,
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battery-free device (FloWatch) in recent years. All have been

proven to be effective in either experimental animals or
humans
Ø Various complications of PAB have been described
ü The band may cut through the PA and fatal bleeding may
occur (rare)
ü the degree of pulmonary constriction may become inadequate
ü Infection at the site of the band is a serious complication
ü development of LVOTO after PAB.
ü Spontaneous closure of VSD after banding
ü Extensive destruction (thrombosis) of the PA
ü Migration of the band toward the bifurcation of the PA may
lead to distortion and narrowing of one or both PAs
ü Infection, possibly with an aneurysm at the site of the PAB is
another possible, serious complication. Airway compression
may also coexist
• Norwood procedure
Ø This is a complex procedure, which includes neo-aorta
reconstruction, aortic arch augmentation and a MBTS or Sano’s
shunt placement
Ø It was designed to treat HLHS, as well as aortic atresia or aortic
stenosis with inadequate LV, mitral atresia or stenosis, IAA and
even DORV with mitral atresia or complete TGA with a hypoplastic
RV.
Mahajan A, Shabanie A, Laks H.

Atrial septostomy Interatrial septostomy under
transesophageal echocardiography
Subjected to different techniques: guidance: a novel approach. J Thorac
1. Blalock-Hanlon operation (atrial septectomy w/o CPB) Cardiovasc Surg. 2002
• Atrial septectomy was done to create an ASD to improve Apr;123(4):824-6.
intracardiac mixing at the atrial level, which then became the Komai H, Naito Y, Fujiwara K,
standard treatment for TGA where the ASD was too small. Uemura S. The benefits of surgical
• In TGA, VSD, the Blalock- Hanlon operation was added when PAB atrial septostomy guided by
was carried out. transesophageal echocardiography in
• In cases of TGA and subpulmonary stenosis, the Blalock-Hanlon pediatric patients. J Thorac
operation could be performed simultaneously or before a systemic- Cardiovasc Surg. 1999
to-pulmonary shunt. Oct;118(4):758-9.
• The Blalock-Hanlon operation was also indicated for palliation of Park SC, Neches WH, Mullins
mitral atresia CE, Girod DA, Olley PM, Falkowski
G, Garibjan VA, Mathews RA,
Fricker FJ, Beerman LB, Lenox CC,
2. Park procedure (balloon or blade atrial septostomy)
Zuberbuhler JR. Blade atrial
• The main indication for this procedure is TGA, aiming at enlarging
septostomy: collaborative study.
the foramen ovale, and thereby allowing some oxygenated blood to
Circulation 1982 66(2):258-66.
pass from the left ventricle into the aorta.
3. Rashkind procedure (balloon catheter atrial septostomy)

• The balloon catheter technique for atrial septostomy was applied
broadly and displaced the surgical atrial septal defect creation
technique by offering acceptable immediate palliation in small
infants with complete transposition of the great arteries and those
unlikely to achieve successful correction
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Atrial switch operation (ASO)

• There are two types of atrial switch operations: the Mustard’s
operation and the Senning’s operation. Both are similar in principle
but differ in technique.
• The atrial switch operation is an open-heart procedure under
cardiopulmonary bypass, during which the right atrium is opened
and the wall between the atria is fully removed. By using the
pericardium (Mustard) or flaps made from the atrial septum and
wall (Senning), a ‘‘baffle’’ is constructed, directing blood from the
veins in the right atrium towards the left ventricle.
• Palliative atrial switch procedures without closure of the ventricular
septal defect have been used in the treatment of deeply cyanotic
patients with severe pulmonary vascular obstructive disease
Other palliative procedures

6. Mitral valve palliation Sources for further reading
• Congenital mitral stenosis requires surgical palliation in small Textbook Chapters
infants in whom currently available prosthetic valves cannot be Chapter 63: Palliative Procedures in
inserted into the mitral annulus. Cyanotic Congenital Heart Disease
• A left atrial-left ventricular conduit bypass is an alternative for the and chapter 64: Pulmonary Artery
relief of congenital mitral stenosis and can obviate the need for Banding. Glenn's Thoracic and
valve replacement in infancy. Cardiovascular Surgery (Baue, Geha,
Hammond, Laks, and Naunheim), 6th
ed., 1073-1094.
7. Aortic valvotomy
Chapter 8: Palliative Operations.
• Aortic valvotomy for the relief of congenital aortic stenosis is a
Pediatric Cardiac Surgery (Mavroudis
palliative operation. and Backer), 2nd ed., 104-114.
• Current therapy for critical congenital aortic stenosis includes either
balloon or surgical valvotomy. Both procedures leave residual
lesions that often require further treatment.
8. Palliation of coarctation of the aorta

• Intravascular stent placement for native and recurrent coarctation
of the aorta has been used successfully in various locations.
9. Hybrid palliation
• Hybrid cardiac surgery was defined as combined catheter based
ductal stenting and surgical interventions such as bilateral
pulmonary artery banding either in one setting or in a sequential
fashion within 24 h.
• Hybrid palliation, which was developed originally for HLHS, is now
applied to patients with a functional single ventricle and restrictive
systemic outflow tract, such as tricuspid atresia with transposition
of the great arteries.
• The primary palliation procedures include stenting of the ductus
arteriosus with a self-expanding nitinol stent to secure adequate
systemic blood flow, placement of an internal pulmonary arterial
band to protect the pulmonary vascular bed and prevent pulmonary
overcirculation and widening of the interatrial communication by
blade and balloon septostomy or static balloon dilation to
decompress the left atrium.
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Pacemaker use in pediatrics

Lead technology
• The lead may be unipolar, in which the tip is the cathode and
the pacing box can is the anode, or bipolar, in which the
anode is an additional electrode just proximal to the tip.
• Unipolar leads are:
Ø smaller
Ø more flexible and can be repaired;
Ø the stimulus artifact is easier to see
Ø may be more reliable.
• Bipolar leads:
10. have significantly better sensing potentials,
11. particularly far-field sensing,
12. avoid local skeletal muscle twitch and skeletal myopotential
over sensing
13. allow submuscular implantations of generators
Pacemaker Terminology
Position I II III IV V
Category Chamber(s) Chamber(s) Response to Programmability Antitachyarrhythmia

paced sensed sensing rate, modulation function(s)
0 = None 0 = None 0 = None 0 = None 0 = None

A = Atrium A = Atrium T = Triggered S = Simple P = pacing
programmable (antitachyarrhythmia)
V=Ventricle V=ventricle I = Inhibited M=Multiprogra- S = Shock

mmable
D = Dual D = Dual D = Dual C= D = Dual (P + S)

(A + V) (A + V) (T + 1) Communicating
R = Rate
modulation
• The fifth letter relates to a pacemaker that has antitachycardia functions
• Examples,
Ø VVIR mode is a ventricular-paced, ventricular-sensed mode that is inhibited by spontaneous
activity and in addition has a rate response sensor;
Ø DDD means that both atria and ventricles are paced and sensed and both inhibited and
triggered.
Implantation In Children
Recommendations for permanent pacing in patients with CHD
Class I
1. Symptomatic advanced second- or third-degree AV block
2. Symptomatic sinus node dysfunction with correlation of symptoms
3. Postoperative advanced second- or third-degree AV block > 7 days
4. Congenital third degree AV block with wide QRS escape rhythm,
complex ventricular ectopy or ventricular dysfunction
5. Congenital third degree AV block in the infant with a ventricular
rate < 50/55 bpm or with CHD and a ventricular rate < 70
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6. Sustained pause-dependent VT
Class IIa
1. Bradycardia-tachycardia syndrome requiring anti-arrhythmic
therapy
2. Congenital third-degree AV block with an average heart rate < 50
bpm or abrupt pauses
3. Long QT syndrome with second- or third-degree AV block
4. Asymptomatic sinus bradycardia with congenital heart disease with
low resting heart rate
5. Congenital heart disease with reduced function due to sinus
bradycardia
Class IIb
1. Neuromuscular disease with any degree of AV block
2. Asymptomatic sinus bradycardia < 40 bpm with congenital heart
disease
3. Asymptomatic congenital third-degree block with an acceptable
rate and narrow QRS complex
Choice Of System To Be Implanted

General principles of pacemaker choice:
1. The ventricle should be paced if there is actual or threatened atrio-
ventricular block.
2. The atrium should be paced/sensed unless contraindicated.
3. Rate response is not essential if the patient is inactive or has a
normal chronotropic response.
4. Rate hysteresis may be useful if the bradycardia is intermittent.
• The reasonably good system will produce a satisfactory long-term

result with minimal battery and lead replacements.
• The more complex dual chamber generators have generally a
shorter lifespan than similarly sized single-chamber generators.
• Bipolar leads, which are most suitable for atrial sensing and pacing,
are larger and stiffer than unipolar leads and two leads may be
difficult to be put into a small vein.
• In addition, the pacemaker syndrome, which occurs with retrograde
ventricular–atrial conduction and causes reflex hypotension in
adults with ventricular pacing, is very rare in children
• Small pacing generators are available for very small children;
however, the disadvantage is a reduced lifespan.
• The new adult-sized generators are usually small enough for use in
the majority of children, except for neonates and very small
infants, particularly if they are implanted deep to the rectus muscle
or in the suprarenal area
Wire Implantation
a) Epicardial implantation
• Indications for epicardial implantation are:
I. Small size (< 8 kg)
II. Right-to-left shunt
III. Venous access difficulties
IV. Postoperative Glenn procedure
V. Superior vena caval obstruction
VI. Ventricular access difficulties
VII. Mechanical tricuspid valve replacement
VIII. Fontan-type operation
IX. Failed transvenous system
• The advantages are that leads do not become displaced
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• The disadvantages are the need for an operative procedure

(often a thoracotomy), somewhat higher initial voltage
threshold, and a higher incidence of lead fracture and late
threshold rise and sensing problems (exit block) related to
fibrosis at the junction between the electrode and the
myocardium. These difficulties have been significantly reduced
with the introduction of a steroid eluting epicardial lead.
a) Endocardial implantation
It is the usual approach because the initial and long-term
thresholds are lower. With fixation at the distal end, either with
tines or screws, an adequate loop can be created in the atria to
allow for growth.
Implantation Techniques
a) Temporary Systems
I. Transvenous
A temporary bipolar system can be inserted transvenously from any
suitable venous access, the optimum being the subclavian vein, from
which the electrode is less likely to displace and the site can be easily
kept clean
II. Epicardial
At the end of most open-heart procedures, two atrial and two
ventricular wires are sewn on. This allows atrio-ventricular sequential
pacing, which is particularly important in patients whose cardiac
output is borderline initially after operation
b) Permanent Systems
1. Endocardial Implantation
The procedure requires X-ray fluoroscopy with a C arm and is
performed in a catheter laboratory or a pacing theatre The subclavian
vein is our approach of choice
2. Epicardial Implantation
Permanent wires can be positioned on the epicardial surface through
a left thoracotomy, also by a subxiphoid or mediasternotomy
approach
Optimal guideline measurements at lead implantation.
Parameter Atrial Ventricular
P–R amplitude (mV) > 1.5 > 4.0
Slew rate (V/s) > 0.5 > 0.5
Threshold (V) at 0.5 < 1.2 < 1.0
ms
Pacing impedance > 500 > 500
(Ohms)
Biventricular Pacing (Cardiac Resynchronization Therapy)

• In association with heart failure, there are electromechanical
abnormalities of prolonged atrio-ventricular, interventricular and
intraventricular delay
• In children the indications are evolving. Some patients with primary
dilated cardiomyopathy with left bundle branch block pattern can
be improved, unless in NYHA Class 4.
• Reversal of ventricular dysfunction secondary to prolonged right
ventricular apical pacing is a very logical indication.
• The necessity for three leads within the venous system in children
makes it suitable only for older children. In smaller children an
epicardial approach has been used.
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Pacemaker Problems
• Electrode Dislodgement
• High Pacing Threshold
• Lead Fracture
• Muscle Twitching
• Erosion
• Left Ventricular Lead Malposition
• Infection
• Non-functioning Wire
Pediatric Cardiac Mechanical Support

Aims of cardiac mechanical support
• Restore adequate oxygen delivery to vital organs
• Provide time and the appropriate physiological milieu for
myocardial recovery
• The aim of mechanical support may change from recovery to a
bridge to heart transplantation
Choice of mechanical assistance device
Currently the two most common modes of short-term cardiac support
used in infants and children are ECMO and centrifugal extracorporeal
VADs.
Comparison between ECMO and extracorporeal centrifugal VAD
ECMO VAD
Oxygenator Yes No
Circuit Longer, with reservoir Short, no reservoir
Anticoagulation ACT 180–220 seconds ACT 140–160 seconds
Risk of air Low (air trapped in Higher, particularly with
embolus the circuit) L-VAD
Type of Cardiorespiratory Cardiac
support
Ventricular May need atrial Not required
decompression septostomy/ LA vent
Length of Up to weeks Up to weeks
support
Cannulation Trans-thoracic or neck Trans-thoracic
Extracorporeal membrane oxygenation (ECMO)

• ECMO remains the mainstay form of mechanical cardiac support
used in the paediatric population.
• There are two principal modes of ECMO support:
• veno-venous ECMO provides only respiratory support, whilst
• veno-arterial ECMO bypasses both the heart and lungs
• Survival-to-discharge rates of patients treated with ECMO for short-
term cardiac support are in the range 40–80%, depending on the
patient’s diagnosis and underlying pathophysiology.
VENTRICULAR ASSISTANCE DEVICES (VADs)

• Extracorporeal support with non-pulsatile centrifugal VADs have
been used to effectively support infants and children with
ventricular failure after congenital heart surgery.
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• Survival to- hospital-discharge rates similar to that of ECMO
Intra-Aortic Balloon Pump (IABP)

• The vast majority of experience with IABP remains in the adult
population and these devices, therefore, cannot routinely be
recommended for infants and small children.
• In the older children and adolescents, the IABP may have a role in
treating patients with moderate left ventricular failure prior to
inserting a different form of mechanical assistance or as an adjunct
for weaning from ECMO.
Indications and timing for mechanical support

The indications for paediatric cardiac mechanical support can be
divided into two groups:
a) Patients with cardiac failure following open heart
surgery:
1. The postcardiotomy myocardial dysfunction is usually related to
myocardial stun, due to myocardial ischaemia–reperfusion
injury, direct myocardial injury from surgery and the
inflammatory response to cardiopulmonary bypass.
2. It is vital to identify any potentially correctable residual
anatomical lesion as the contributory cause of the patient’s
circulatory failure.
3. Other, less common indications include refractory pulmonary
hypertension and malignant arrhythmias
4. There is no easy formula for predicting severity of illness and
patient’s outcome as there is for respiratory ECMO using the
oxygenation index
5. Because of the concerns of myocardial damage associated with
high doses of epinephrine and to avoid organ damage from
periods of low cardiac output, in most centres, postoperative
mechanical support is initiated in patients with worsening signs
of inadequate oxygen delivery (rising lactate, falling mixed
venous oxygen saturation, wide core–peripheral temperature
gap, poor urine output and other signs of end-organ ischaemia)
despite appropriate vasoactive medical management.
6. As an indication for mechanical support, there is now increasing
evidence that patient’s outcome is related more to the potential
reversibility of myocardial dysfunction than to the underlying
cardiac anatomy.
b) Medical patients with cardiac failure due to a
spectrum of diseases, including myocarditis,
cardiomyopathy, pulmonary hypertension,
arrhythmias, and events related to cardiac
catheterization and infants who decompensate
prior to surgery.
• It seems more appropriate that these patients with acute
cardiac failure are supported in the first instance as a bridge
to recovery.
• When choosing the optimal time for initiation of mechanical
support for these patients, the same principles
• apply as to postcardiotomy patients. Occurrence of
arrhythmias, intolerance of enteral feeding and oliguric
• renal failure are often ominous signs for semi-elective
initiation of mechanical support
• In patients who fail to show improvement in ventricular
function over the course of the first week the aim of support
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should be reviewed as a bridge to transplantation
Patient selection criteria •

The criteria for mechanical support have continued to evolve with the
increasing ECMO experience and the development of new mechanical
devices. It is therefore important to focus on the principles of patient
selection, rather than absolute criteria:
• First, the patient is rapidly deteriorating despite maximum
conventional treatment.
• Second, the underlying cardiac and/or respiratory pathophysiology
is potentially reversible, unless the aim of support is as a bridge to
heart transplantation.
• Third, any haemodynamicaly important, surgically correctable,
residual lesions should be identified, as the presence of such
unrecognized defects may exacerbate the haemodynamic
compromise and minimize the chances of recovery. It is crucial that
patients have detailed postoperative imaging, either by
echocardiography, cardiac catheterization, computed tomography
or magnetic resonance scan.
• Fourth, the patient should not have suffered any other complication
for which intensive care support would otherwise be discontinued
(such as severe neurological injury from hypoxic–ischaemic
encephalopathy) and/or does not have an absolute contraindication
to anticoagulation (such as a recent significant cerebral
haemorrhage).
• From a practical point of view, it is often impossible in the
emergency setting to adequately assess the patient’s cerebral
function. In this circumstance the patient is given the benefit of
doubt and the neurological function tests are carried out as soon as
feasible on mechanical support.
Complications of mechanical support
1. Surgical site bleeding (incidence ± 30%).
2. Infection (incidence ± 10%; associated with increased length of
time on the device).
3. Neurological events, i.e. haemorrhage and infarcts (incidence ±
6%; higher in neonates at ± 12%).
4. Oxygenator, pump or circuit failure (incidence ± 10%).
Practical aspects of mechanical support

• Veno-arterial ECMO in children can either be instituted via
cannulation of the neck vessels, femoral vessels, or cannulation via
a median sternotomy, usually through the operative site
• Left ventricular cannulation is now thought to be the preferred site,
as it is associated with improved drainage and decompression of
the left ventricle, which may lead to lower rates of cerebral events
• It is vital to decompress the left heart, particularly where there is
marked left ventricular impairment and failure of aortic valve to
open. In this circumstance the left atrial pressure is likely to be
very high (above 30 mmHg), causing lung congestion. A high left
ventricular end-diastolic pressure is also likely to increase
ventricular wall stress and impair coronary perfusion. The
decompression of the left heart can be achieved either by creating
an atrial communication in the catheter laboratory or by directly
placing a left atrial vent.
• Excessive bleeding is one of the most common complications of
postoperative cardiac ECMO. The management of postcardiotomy
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bleeding involves:
• Aggressive correction of any coagulopathy (maintaining platelet
count > 100 000 with platelets transfusion) and fibrinogen > 2 g/l
with cryoprecipitate.
• Maintaining the activated clotting time (ACT) in the range 160–180
seconds.
• Administration of antifibrinolytic agents, such as aprotinin.
• Chest re-exploration for significant ongoing bleeding (> 10
ml/kg/hour) despite correction of the patient’s coagulopathy and
the above mentioned manoeuvres.
• Conversion of chest to neck cannulation if applicable.
• Using a blood cell-saver.
• If the bleeding continues, running the circuit heparin free for a
period of 4–6 hours until the bleeding is controlled. In this situation
a spare primed ECMO circuit should be readily available on stand-
by in case of major clotting of the circuit.
• More recently, the use of recombinant activated factor VII has been
advocated for refractory bleeding in postcardiotomy patients, yet,
cannot be recommended for routine use until its safety and efficacy
has been tested in randomized clinical trials
• In patients after Norwood stage I operation with BT shunt, VAD
support is preferable to ECMO, unless there is an evidence of
associated lung injury. The VAD flow should be maintained high (at
around 200 ml/kg/min) to compensate for the patent shunt and to
ensure adequate systemic perfusion. When ECMO is used for these
patients, the BT shunts should not be fully occluded, as this can
result in shunt thrombosis and infarction of the lungs
• Patients should be managed in the most physiological manner
possible, i.e. enterally fed (unless contraindicated) and minimally
sedated to keep them comfortable and safe
• Ventilation on ECMO is usually maintained on ‘‘resting’’ settings,
with tidal volumes of 5–10ml/kg (maximum peak inspiratory
pressure 25 cmH2O), PEEP 7–10 cmH2O and a ventilator rate of
15–20/min. In order to avoid pulmonary venous desaturation, the
FiO2 on the ventilator is typically set at 40%. In contrast to ECMO,
full ventilation is mandatory in patients on VAD.
• Although antibiotic cover is not routinely necessary for patients on
ECMO, it is common practice to perform daily blood cultures and
treat any new infection aggressively.
Residuae, Sequalae & Complications

There might be differences of opinion among cardiologists and
cardiovascular surgeons whether an abnormality identified after heart
surgery constitutes a residual defect, a sequel of the operation, or a
complication of the repair. Thus, the usage of these terms requires
definition.
The term postoperative residua refers to any of the remaining

anatomic and hemodynamic abnormalities which existed as part of
the CHD or those which developed or could develop as a consequence
of the defect. The residua in some instances might eventually be
preventable in future patients by improvement in diagnostic or
surgical techniques or by closer attention to those techniques
currently available. In other instances, the nature of the malformation
is such that a residual abnormality either could not be corrected or
correction was not worth the added risk to the patient. It may be the
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case that the residua was located away from the area of primary
operative exposure and no attempt was made to approach the
additional defect surgically. The likelihood of certain defects
remaining after surgery is unpredictable, while the chance that others
will persist despite an otherwise successful operation can be
anticipated quite accurately.
The term sequelae will be used to refer to those anatomic and

hemodynamic consequences of the operative procedure which are
apparently unavoidable in our current state of knowledge. However,
once the method of avoiding an important 34equel has been
developed, its continued occurrence constitutes a complication as
defined in the following section. Some sequelae, whether clinically
obvious or not, are inconsequential, while others lead to manifest or
potential problems. A few of the sequelae may be more devastating
than the original circulatory problem for which surgery was
performed.
Complications are unexpected events related to the surgery though

their occurrence is not always without precedent. Complications do
not necessarily imply a mistake in judgment or performance on the
part of the cardiologist, anesthesiologist, nurse, or surgeon, but
certainly errors, however excusable, do sometimes result in
complications. Some of the complications to be discussed are
currently unpreventable.
Prognostic Considerations
Risk scores of CHD
Refer to Aristotle basic and modified scores.
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35
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45
Appendix I: Pediatric Cardiology Milestones – Timeline
Selected Milestones in Pediatric Cardiology
1628 - William Harvey published De Motu Cordis - first description of 2 circulations (pulm & syst.)
1671 - Neils Stenson first describes anatomy of TOF - Copnhagen
1676 - Thomas Sydenham first describes rheumatic fever
1715 - Vieussens first describes rheumatic mitral and aortic valve disease on autopsy
1819 - Laennec invents the stethescope, & physicians relate murmurs to each heart disease
1888 - Etienne-Louise Fallot descrbes TOF further, as a major cause of cyanotic heart disease
1889 - Cheadle describes children's cardiac disease after sustaining rheumatic fever
1896 - WC Roentgen develops the X-Ray
1896 - FH Williams first describes using a fluroscope to define the heart border
1902 - Einthoven develops the ECG
1907 - Maude Abbott publishes her atlas of CHD
1929 - William Forsmann passes a cath from his own arm to his RA
1932 - Richardson & Cournard start using cardiac catheterizations for diagnostics
1938 - (August 26) - Robert Gross ligates PDA - 7yo F - Boston
1939 - Bing reports angiography use for CHD diagnosis - Cuba
1944 - Helen Taussig, Alfred Blalock, Vivien Thomas creates the classic BTS- LSCA to LPA anast. for TOF
1945 - Clarence Crayfoord & Gustave Nylin - correct CoAo with end-end anast - Stockolm
1948 - Russell Brock relieves pulmonary stenosis with a transventricular approach
1953 - John Gibbon closes an ASD with the first use of a CPB machine - 18yo F - Philadelphia
1953 - Rubio-Alvarez incises a pulmonary valve by pulling a wire through it - the first therapeutic cath
1954 - C Walton Lillehei - first use of crossed-circulation as CPB for VSD, TOF, AVCD - Minneapolis
1954 - Edler & Hertz first describe the use of reflected ultrasound to image the heart
1955 - Kirklin first uses a modified version of Gibbon's CPB machine - Mayo Clinic
1958 - Donald Heath & Jesse Edwards first report on pulmonary hypertension, informing why certain
patients did poorly after VSD repair
1958 - Maurice Lev describes the conduction system in CHD pts, thus limiting risk of heart block, a major
complication - Chicago
1959 - Senning reports first successful repair of TGA w atrial switch
1961 - Pediatric Cardiology becomes the first boarded pediatric sub-specialty
1964 - Mustard first successful repair of TGA w atrial switch w an intra-atrial baffle
1964 - CT Dotter & MP Judkins report use of sequential dilators to dilate peripheral arteries, introduced via
surgical cutdown
1966 - Williams Rashkind performs balloon atrial septostomy to palliate TGA
1966 - Jean Kan performs balloon valvuloplasty to open a pulmonary valve
1967 - Kawasaki first reports mucocutaneous lymph node syndrome
1967 - Porstmann reports first PDA plugging
1960s - Giancarlo Rastelli develops new classification for CAVSD with subsequent better results, along
with the Rastelli approach for TGA+VSD+PS - Mayo Clinic
1971? - Fontan develops the cavopulmonary anastomosis for tricuspid atresia palliation - Paris
1973 - Coceani & Olley report that PGE1 dilates the DA in lambs
1974 - King & Mills first close an ASD via cath, using a double-disk
1974 - Gruntzig & Hopff report the ise if balloons to dilate peripheral vessels
1976 -Adib Jatene first repairs TGA with an anatomical repair, switching GAs w cor arts - San Palo, Brazil
1970s (late) - M-mode first becomes useful for cardiac dx - check PDA closure, LA &LV size, vlv gradients
1980s - 2D echo & color Doppler become available
1981 - Singer first uses a balloon to re-dilate a repaired coarctation of the aorta
1983 - William Norwood develops the Norwood palliation for HLHS
1984? - Aldo Casteneda first does TGA arterial switch in the newborn - Boston
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Appendix II: Surgical Eponyms

RSVC to RPA - Classic Glenn (1950s)
-bidirectional Glenn = SVC to RPA w Q to both lungs
Classic BT Shunt- subclavian to PA, with loss of Q to the ipsilateral arm

Modified BT Shunt- R innominate to RPA w Gortex (usually 3.5mm)
Waterston Shunt- Asc Ao to RPA direct anastomosis- grows w pt, so no resistor, so much incr Qp w growth
Waterston Cooley- Asc Ao to RPA w Dacron or Gortex - allows for more resistance so less Qp w pt growth
Potts- LPA to Desc Ao - in pts w Eisenmenger syndrome- less likely to have parodoxical emboli to brain than a
Waterston shunt
Mee (Cental) Shunt- MPA to Aorta (fr confluence of PAs) w prosthetic material or direct anastomosis
TGA Repair
Atrial Switch - switch w baffle at atria
-Mustard- uses artificial material (Dacron/Gortex)
-Senning- uses RA/LA wall instead of artificial material (harder to perform)
-xx = large atrial scar w IART
Arterial Switch
-switch the GAs
-able to be done now bc we move the coronaries...
-Lecompte Maneuver- PA is entirely anterior to Aorta (instead of LPA going underneath the Ao Arch)--> risk for prox
PA branch stenosis
Yacoub- classification of coronaries in TGA
HLHS
Norwood- started Norwood procedure in 1982
-Neoaorta created
-separate pulm and systemic Q
-usually combine with a BT shunt or Sano/Brawn
Sano- RV to LPA
Brawn- RV to RPA
Hybrid- stent the PDA and band the PA
-advantage of Sano/Brawn- less hypoperfusion during diastole...
Damus Kaye Stansel

-direct anastomose of proximal PA to Asc Ao to ensure Q to Ao (for situation of Ao obstruction)
ddx fr Norwood- no Ao reconstruction, and here you put PA to side of Ao, not Ao to side of PA...
Glenn- SVC to PA
-bidirectional done currently (unidirectional done in past)
Fontan- SVC and IVC to PA - first done in 1968
-+/-extracardiac
- +/- fenestration
Pulmonary Atresia with VSD

-MAPCAs- Major Aorto Pulmonary Collateral Arteries- seen w pulm atresia/severe PS to supply Qp
-?enlarged bronchial arteries
-come fr Desc Ao usually, but also fr arch, subclavian, coronaries
-sometimes must grow the PA's before attaching them to a graft connecting PAs to the RV
-Contegra bovine jugular graft- use a jugular venous valve
Rastelli-
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-(ddx Rastelli Classification of AV canal defects)

-Tx dTGA + VSD + PS
-tunnel LV to Ao with a baffle, closing the VSD, and place an RV to PA conduit
-xx- may have obstructed conduit, baffle leak, and later need conduit change as pt grows
Nikaidoh
-harvest Ao root fr RV (may move coronaries too), relieve LVOTO by dividing outlet septum and excising pulm vlv,
and reconstruct LVOT, anastomose PA to RV, and Ao to LV... --> free Pulm regurg
-includes a Lacompte too
REV Procedure = Reparation a l'Etag VEntriculare

-Ao connect to LV via the VSD, like the rastelli
-remove conal septum, pulm trunk directly reimplant to RV w/o conduit, and do a Lacompte
--> avoid subAo stenosis, late ventric deterioration, and arrhythmia/sudden death
ccTGA
-might not do anything
-might do Double Switch- mustard/senning + arterial switch
Ross Procedure
-excise pulm valve and implant it as a neoaorta valve
-use RV-PA conduit for Qp
-must move coronaries to neoaorta...
Yasui
= Norwood-Rastelli
-Aortic Atresia/LVOTO with a VSD, but normal MV annulus size and normal LV size
-LV fx and size good enough for systemic circulation (is apex forming)
-Repair Aorta, and connect to PA w a DKS, then use an RV to PA conduit and close the VSD w a baffle
(ddx fr Sano bc here you get 2 ventricles)
Bentall Procedure
-indication- combined asc ao and ao vlv dz- ao aneurysm/dilation, AR, Ao dissection
-replace the asc ao and valve with a graft and replace the cor arts into the graft
-Alternative: Valve sparing root replacement- keep the Ao vlv
Konno
-Aortoventriculinfundibuloplasty
-used fro complex LVOTO
-initially used to tx severe subAo stenosis...
-originally a patch enlargement of LVOTO and RVOTO and insert mechanical Ao vlv prosthetic
-then = multilevel LVOTO espec if small Ao annulus
-Now- Modified Konno - make a VSD and then patch it to open the LVOTO; often done w a Ross for pts w Ao vlv
severe stenosis
Warden
-for Partial Anom Pulm vn Return w R PVns to SVC near RA jct, along with a Superior Sinus Venosus ASD
-connect RA appendage to SVC to transect it and use it to baffle the SVC inflow to LA and then anast the SVC to the
RA...
47
03 04
Acyanotic Cyanotic
w L®RS PBF
02 05
Acyanotic Cyanotic
w/o Shunt ¯PBF
R. Side
01 06
Acyanotic Cyanotic
w/o Shunt PAH
L. Side
ezzeldinmostafa.com
48
Dr. Ezzeldin Mostafa emostafa@med.asu.edu.eg 2. Acyanotic w/o Shunt, L. sided Lesions
2 Acyanotic, without Shunt, L-sided Lesions

Table of Contents
Supravalvular Aortic Stenosis................................ 8
2 Acyanotic, without Shunt, L-sided Hypertrophic Cardiomyopathy ............................. 11
Lesions ...........................................................2 Aortic arch obstruction ......................................... 14
LVOTO................................................................... 4 Vascular Rings – Slings....................................... 18
Aortic Valve Stenosis ............................................. 5 Congenital Mitral Valve malformations ................ 22
Aortic root enlargement surgery ......................... 6
Subvalvular Aortic Stenosis ................................... 7
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(CHD)
terminology
om/pedscards-
Timeline
Learning Objectives: Selected Articles

Brown JW, Stevens LS, Holly S,
Robison R, Rodefeld M, Grayson T,
LVOTO Marts B, Caldwell RA, Hurwitz RA,
• LVOTOs include a series of stenotic lesions starting in the anatomic Girod DA et al. Surgical spectrum
LVOT and stretching to the descending portion of the aortic arch. of aortic stenosis in children: a
• Obstruction may be subvalvar, valvar, or supravalvar and may thirty-year experience with 257
present alone or in concert, as in the frequent association of a BAV children. Annals of Thoracic
with CoA. Surgery 1988;45:393-403.
• All of these lesions èé afterload on LV and, if severe and This large series reviews the
untreated, èLVH èdilatation and failure of the LV. spectrum of disease in the
pediatric population. There is a
• All patients with LVOTO are considered at a high risk for developing
good discussion of operative
IE, and should always receive appropriate measures for techniques for subvalvular,
prophylaxis. valvular, and supravalvular
• Manifestations depend on severity of stenosis but typically involve stenosis.
pressure overload proximal to the stenosisèheart failure, and van Son JA, Reddy VM, Black MD,
hypoperfusion distal to the stenosis. Rajasinghe H, Haas GS, Hanley
• Severe stenosis (e.g critical AS, severe CoA) can manifest in the FL. Morphologic determinants
neonatal period with acidosis, renal insufficiency, and shock and favoring surgical aortic
may require prostaglandin E1 infusion to reopen the constricted valvuloplasty versus pulmonary
autograft aortic valve replacement
ductus arteriosus as a life saving measure till urgent intervention is
in children. Journal of Thoracic
done. and Cardiovascular Surgery
• Correction is either surgical or by balloon angioplasty ± stent 1996;111:1149-57.
placement. The nice operative photos provide
HCM detail for the discussion on
• HCM is usually due to one of numerous genetic mutations that valvuloplasty. The authors
cause various types of ventricular hypertrophy that restrict filling suggest using autografts for
(ie, cause diastolic dysfunction) and sometimes obstruct LV valvuloplasty failure.
outflow. Douville EC, Sade RM, Crawford
FA Jr, Wiles HB. Subvalvar aortic
• Coronary blood flow may be impaired even in the absence of
stenosis: timing of operation.
coronary artery atherosclerosis because capillary density is
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inadequate and the intramyocardial coronary arteries are narrowed Annals of Thoracic Surgery
by intimal and medial hyperplasia and hypertrophy 1990;50:29-34.
• At a young age, patients may have chest pain, dyspnea, This series of patients covers the
spectrum of subvalvular aortic
palpitations, syncope, and sometimes sudden death, typically
stenosis and various operative
triggered by exertion.
procedures are utilized. The
• Echocardiography is done, but, if available, MRI best shows the authors recommend prompt
abnormal myocardium. operation at the time of diagnosis.
• Use β-blockers and/or rate-limiting Ca channel blockers Sharma BK, Fujiwara H, Hallman
(usually verapamil) to decrease myocardial contractility and slow GL, Ott DA, Reul GJ, Cooley DA.
the heart rate and thus prolong diastolic filling and decrease Supravalvar aortic stenosis: a 29-
outflow obstruction. year review of surgical
• Avoid nitrates and other drugs that decrease preload (eg, diuretics, experience. Annals of Thoracic
Surgery 1991;52:1031-9.
ACE inhibitors, angiotensin II receptor blockers) because these
An extensive series from Texas
decrease LV size and worsen LV function.
which demonstrates the evolution
• Consider an implantable cardioverter-defibrillator for patients with of operative technique for
syncope or sudden cardiac arrest. supravalvular lesions. Excellent
Vascular Rings-Slings diagrams provide visualization for
• Vascular rings-slings are malformation of the aortic arch of variable the various approaches.
surgical anatomy. The most common of which is double aortic arch.
• The diagnosis is based on symptoms related to tracheal and/or Sources for further reading
esophageal compression. Barium esophagogarm is the classic Textbook Chapters
Chapter 32: Congenital Aortic
investigation.
Stenosis. Cardiac Surgery (Kirklin
• Management is surgical division of the ductus arteriosus or
and Barratt-Boyes), 2nd ed.,
ligamentum arteriosum, or the compressing vessel with or without 1195-1238.
remplanatation based on anatomy. Thoracotomy incisions are Chapter 75: Congenital
satisfactory approach in most of lesions. Malformations of the Aortic Valve
Congenital Malformations Of The Mitral Valve and Left Ventricular Outflow Tract.
• Different congenital malformations may affect the mitral valve Glenn's Thoracic and
either in isolation or in association with other cardiac anomalies Cardiovascular Surgery (Baue,
• An accurate description of the malformations can be achieved Geha, Hammond, Laks, and
Naunheim), 6th ed., 1221-1242.
through echocardiography but requires prior knowledge of these
lesions. Thus, the mitral valve should be analysed as an entire
complex, including the valvar leaflets, tensor apparatus and
papillary muscles.
• Successful management of congenital mitral valve disease is closely
dependent on the preoperative assessment of the anatomical
substrate.
• Although mitral valve replacement appears to provide acceptable
mid- and long-term results, mitral valve repair is always preferable
when possible.
LVOTO
Pathophysiology
Physiologic consequences involve 2 phenomena:
Ø Pressure overload in front of LV.
Ø Hypoperfusion effects.
Pressure overload causes left ventricular hypertrophy with
subsequent LV systolic and diastolic dysfunction, increased LA
pressure, left-to-right shunting across PFO, RV volume overload and
increased flow across PDA
Hypoperfusion distal to the obstruction affects all body organs
especially brain and abdominal organs with increased risk for
ischemic complications. In addition, increased LV muscle mass with
coronary hypoperfusion may lead to myocardial ischemia with
eventual subendocardial fibroelastosis.
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Clinical presentation
If LVOTO is significant, circulatory shock with renal insufficiency
(oliguria or anuria) and metabolic acidosis may develop in the first 7
to 10 days of life and may mimic findings of other systemic disorders
such as sepsis. Infants with critical obstruction are likely to become
acutely ill as soon as the ductus arteriosus constricts or closes.
Less severe cases may be asymptomatic during infancy. Subtle
symptoms (eg, headache; chest pain, fatigue, and leg claudication or
syncopal attacks during physical activities) may be present as
children age. Upper-extremity hypertension, in cases of aortic arch
obstruction, is often present. Heart failure rarely develops after the
neonatal period. Rarely, intracerebral aneurysms rupture, resulting
in subarachnoid or intracerebral hemorrhage.
Typical physical examination findings include faint pulses and low
arterial BP according to severity. However, in aortic arch obstruction,
findings include strong pulses and hypertension in the upper
extremities, diminished or delayed femoral pulses, and a BP
gradient, with low or unobtainable arterial BP in the lower
extremities. A grade 2 to 3/6 ejection systolic murmur is often
present at different areas according to level of LVOTO.
Diagnosis
• Chest x-ray and ECG
• Echocardiography with color flow and Doppler studies establishes
the diagnosis.
• CT or MR angiography in older patients with a suboptimal
echocardiographic window
Aortic Valve Stenosis

Definition
Obstruction of the LVOT secondary to aortic valve pathology is
considered the most common form of congenital heart disease
Morphology
a) The aortic valve cusps are dysplastic and thickened. There are
three types According to number of cusps:
· Unicuspid valves: The orifice is usually small and shaped like a
teardrop, with the wide portion in the center of the valve. it's the
most common cause of neonatal critical AS
· Bicuspid valves: it's seen in about two thirds of patients.
. Tricuspid valves: it's the most unusual form.
· All valves have a rudimentary raphe and 3 interleaflet triangles
b) The ascending aorta is often dilated, particularly in its anterior
portion, due to the turbulence associated with ejection through the
stenotic valve or probably due to structural abnormality
of the aortic wall and the turbulence could be a contributing factor.
c) The ascending aorta frequently has an area of intimal thickening a
short distance above the valve related to the jet from the stenotic
valve. This is not usually the site for the development of infective
endocarditis. The infection most frequently involves the upper
surface of the valve cusps or the adjacent aortic sinus.
d) Other associated congenital heart defects
· Concurrent left sided lesions (e.g. SAM, coarctation)
· Patent ductus arteriosus
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· Ventricular septal defect
Management
In cases of severe stenosis (peak instantaneous Doppler velocity of
≥4 m/s) who are symptomatic or have left ventricular systolic
dysfunction:
• Percutaneous interventions; if stenosis is secondary to bicuspid
commissural fusion.
• Surgical repair (valvuloplasty)
• Surgical replacement: bioprosthetic valves or Mechanical
prostheses (superior durability), and Ross procedure
Surgery should also be considered in those with less severe stenosis
who have concomitant moderate or severe aortic valve regurgitation
or a dilated ascending aorta (≥45 mm).
Aortic root enlargement surgery
• Nicks technique: An incision through the midportion of the

noncoronary cusp (NCC) across the anulus down to the upper part
of anterior mitral leaflet (AML). This V-shaped incision is then
patched and ®- ~ 3-mm two sizes larger . The width of patch is
calculated by multiplying the desired increase in diam. by p and
adding 8 mm for suturing.
• Manouguian Seybold-Epting technique: (Patch enlargement of
aortic valve ring by extending aortic incision into AML): A more
radical incision down the commissure between LCC & NCC and
deeply into AML. This allows a greater enlargement of aortic root
(10 - 25 mm. increase). However, the possibility of subsequent
functional impairment of AML, cannot be excluded with this
method when applied for small children.
• Konno-Rastan aortoventriculoplasty: (Especially in children
with hypoplastic LVOT): The aortotomy extends inferiorly across
the anulus and divides the intervent. septum. The incision is then
extended to cut across RVOT as well, resulting in the creation of a
large VSD. A Z-layered patch is used to bridge the defect in the
septum, allowing an adult-sized prosthesis to be sewn in this
greatly expanded aortic anulus. The 2˚ layer of the patch is used
to repair RVOT.
• Bilateral aortic anular enlargement (Yamaguchi technique):
The aorta is opened by a fibusrcated aortotomy (Limited partial
Konno incision in the L. limb of the incision and limited partial
Nicks incision in the R. limb of the incision). Insertion of a
bifurcated tear drop-shaped Dacron fabric lined with a porcine
pericardium. It is for insertion of a prosthesis 4 sizes larger than
the native anulus. It entails no risk of distorting the mitral valve,
damaging the conduction tissue, the main coronary arteries or
their important trunks, or of leading to LV dysfunction. Acceptable
surgical results have been reported with this technique, however,
injuries of important septal arteries and of the conduction system
may occur.
• Norman technique: Valved conduit (Aortic LV apical
conduit): Hypoplastic anulus + previous operation for congenital
AS + unreconstrutable. A valved conduit can be inserted in the LV
apex and jointed to abdomen (supra-renal) or descending thoracic
aorta ± CPB (can be done safely without bypass). Thus creating a
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double outlet left ventricle (DOLV).

• Translocation technique with reimplantation of coronaries:
Small LVOT or destroyed by SBE ± Poststenotic dilatation of aorta.
Implantation of a large-sized prosthetic valve more distally in the
aorta. The native coronary ostia would be oversewn and
saphenous vein grafts (SVGs) attached distal to the valvalar
prosthesis to both RCA and LCA.
• Supraanular tilted (to NCS) position: Insertion of the valve by
Tilting Technique: This method included allowing the R. portion of
the aortic incision to end about 0.5 cmm above the NCS, the use
of simple interrupted sutures, placement of the prosthetic sewing
ring on top of the annulus of the NCS, thereby tilting the valve
slightly in the outflow tract, and routine orientation of the major
opening of the valve toward the anulus of NCS (eg. B.S. valve).
This orientation resulted in the largest EOA at postop.
catheterization
• Ross technique (Total root replacement with an aortic or
pulmonary allograft and coronary reimplantation): It is the
preferred technique for infants and children with hypoplastic aortic
valve and tunnel subaortic stenosis ± Konno operation (Ross-
Konno procedure).
• Manoguian and Seybold-Epting technique (Patch enlargement of
the aortic and mitral valve rings with DVR)
• Kitamura's technique (The coupling valve Graft): The
coupling valve graft technique is more better. Since making the
coupling graft prior to the operation may save time and may also
make the technique easier by allowing the 2 prostheses to be
sutured in block. In preparing the coupling valve graft, it is
important that the distance bet. the 2 prosthetic valves be fixed at
about 5 mm and that the length of contact bet. the valve and graft
be 10 mm if the valve size is to be enlarged by two sizes.
Since combinations of the 2 prosthetic valves are assumed to be fixed, it is possible to prepare the
coupling valve graft prior to the operation It would be possible to cope. with any operation with use of
23 mm (A) & 29 mm (M), and 25 mm (A) & 31 mm (M).
Subvalvular Aortic Stenosis

Definition
Obstruction of the LVOT below the aortic valve is present in about
20% of patients with LVOTO. It is associated with a variety of other
cardiac anomalies in 2/3 of patients.
Morphology
a) Localized form, also known as discrete fibromuscular ring
· Obstruction occurs at a point between the aortic valve and the
anterior leaflet of the mitral valve
· Seldom presents before 6 months and it may be acquired and is
associated with many other lesions.
b) Diffuse form, also known as tunnel, muscular, or HOCM· It is most
commonly associated with other anomalies such as the Shone complex
· It is spectrum of defects with a variety of etiologic factors; however,
the idiopathic type appears to be genetic
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Management
Indications:
1. Severe SAS (resting catheter-determined or Doppler-derived
estimated peak instantaneous pressure gradient of ≥50 mm Hg)
2. Lower gradients (peak instantaneous pressure gradient <50 mm
Hg) if there is left ventricular systolic dysfunction,
moderate/severe aortic regurgitation, or a VSD.
3. Development of symptoms attributable to SAS (angina, dyspnea,
or syncope) on exertion
Treatment:
a) Surgical resection is the intervention of choice and is usually done
via a transaortic approach.
Surgical management consists of:
Focal SAS: discrete membrane excision and/or blunt dissection with
focal septal myomectomy.
Tunnel-type SAS: more surgically challenging and often necessitates
concomitant myomectomy or application of the Konno-Rastan
procedure to reconstruct the LVOT.
Concomitant repair of the aortic valve is performed if aortic
regurgitation severity is more than mild.
Results:
Surgical mortality is low, and complications are generally minimal.
SAS recurs in up to 37% of cases after surgical resection. The
presence of an immediate postoperative gradient of >10 mm Hg led to
progressive recurrent SAS in 75% of patients; therefore, considerable
attention must be paid by a qualified surgeon to the excision of all
abnormal tissue to include myomectomy of the base of the membrane
and removal of the membrane from the anterior mitral leaflet. Time to
recurrence depends on SAS type; the tunnel-type lesions recur earlier
than the discrete lesions.
Progressive aortic regurgitation may develop despite relief of SAS due
to persistent turbulent flow patterns in the LVOT after SAS resection
that may continue to cause valve damage. Therefore, although
surgical resection is the treatment of choice for this disease, the
optimal timing for surgery can be elusive
b) Percutaneous balloon dilation of a fixed focal stenosis causes short-
term improvement in the gradient and may be considered for palliation
of SAS.
Supravalvular Aortic Stenosis

Definition
Obstruction of the LVOT occurs at the superior margins of the sinuses
of valsalva. It's infrequent form of LVOTO. 30-50% of these individuals
have Williams' syndrome, however, it may be sporadic or familial.
Morphology
a) It's either localized or diffuse and symmetric to asymmetric.
b) It is often associated with degenerative coronary artery disease
c) Characteristics
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· Complete anomaly of aortic root

· Narrowing of sinus rim
· Thickening of media and intimal hyperplasia
· Coronary ostia may be obstructed
· Aortic valve cusps involved (thickened)
· Aortic valve cusps normal length (buckled)
· Associated subvalvular LVOTO
Management
a) Surgical management is recommended with symptoms (angina,
dyspnea, syncope) or a mean pressure gradient of ≥50 mm Hg.
It consists of:
• Excision of a focal stenosis with end-to-end anastomosis of the
ascending aorta,
• Patch enlargement of the sinotubular junction, or more
complex aortoplasty involving patch placement into ≥2 sinuses
of Valsalva.
• The Ross procedure has also been used to replace the aortic
root in patients with concomitant aortic valve disease.
• Aortic valvuloplasty or valve repair in patients with SVAS and
BAV regardless of the presence of stenosis or regurgitation.
b) Balloon angioplasty of SVAS is not useful.
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FIGURE 2−6. Algorithm for the management of LVOTO (supravalvar, valvar, and
subvalvar).
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Hypertrophic Cardiomyopathy
Definition
Hypertrophic cardiomyopathy (HCM) is a congenital or acquired
disorder characterized by marked ventricular hypertrophy with
diastolic dysfunction but without increased afterload (eg, from
valvular aortic stenosis, coarctation of the aorta, systemic
hypertension).
HCM is a common cause of sudden death in young athletes. It may
cause unexplained syncope and may not be diagnosed before
autopsy.
Pathophysiology
A. Etiology
• Inherited: most of cases of with at least 50 different
mutations are inherited in an autosomally dominant pattern.
• Acquired: rare cases. It may develop in patients with
acromegaly, pheochromocytoma, and neurofibromatosis.
B) Morphology
• Asymmetric septal hypertrophy: most common form. It
affects either:
Ø The upper interventricular septum below the aortic valve with
little or no hypertrophy of the left ventricular (LV) posterior
wall; this pattern appears to accelerate during puberty. It's
sometimes associated with systolic anterior motion of the
mitral valve. The resulting disorder may be termed
hypertrophic obstructive cardiomyopathy.
Ø Less commonly, midventricular hypertrophy leads to an
intracavitary gradient at the papillary muscle level. In both
forms, the distal LV may ultimately thin and dilate.
Ø Apical hypertrophy can also occur but does not obstruct
outflow, although it may obliterate the apical portion of the
LV during systole.
• Symmetrical and diffuse hypertrophy.
The myocardium is abnormal with cellular and myofibrillar disarray,
although this finding is not specific for HCM.
C) Hemodynamic consequences
• Contractility is grossly normal, resulting in a normal ejection
fraction (EF).
• Hypertrophy results in a stiff, noncompliant chamber (usually the
LV), elevating end-diastolic pressure and thus increasing
pulmonary venous pressure. As resistance to filling increases,
cardiac output decreases, an effect worsened by any outflow tract
gradient or tachyarrhythmias.
• Coronary blood flow may be impaired, causing angina pectoris,
syncope, or arrhythmias in the absence of coronary artery disease
(CAD). It may be due to:
a. Capillary density relative to myocyte size is inadequate
(capillary/myocyte imbalance)
b. Narrow lumen of intramyocardial coronary arteries by intimal
and medial hyperplasia and hypertrophy.
An effect worsened by exercise that lowers peripheral vascular
resistance and aortic root diastolic pressure, thus reducing coronary
perfusion pressure.
Impaired coronary perfusion may cause chronic diffuse ischemia with
subsequent myocytes death and replacement by diffuse fibrosis.
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Then, the hypertrophied ventricle with diastolic dysfunction gradually

dilates and systolic dysfunction also develops.
• HCM can be complicated by infective endocarditis because of
the mitral valve abnormality or the rapid blood flow through
the outflow tract during early systole.
Atrioventricular block is sometimes a late complication.
Typically, symptoms appear between ages 20 and 40 and are
exertional.
Dyspnea, chest pain, palpitations, and syncope.
Syncope (usually on exertion) either because outflow obstruction or
nonsustained ventricular or atrial arrhythmia. Syncope is a marker of
increased risk of sudden death, which is thought to result from
ventricular tachycardia or fibrillation.
BP and heart rate are usually normal.The carotid pulse has a brisk
upstroke, bifid peak, and rapid downstroke.
The apex beat may have a sustained thrust due to LV hypertrophy. A
4th heart sound (S4) is often present and is associated with a
forceful atrial contraction against a poorly compliant LV in late
diastole.
Systolic ejection murmur on the left sternal border in the 3rd or 4th
intercostal space. It can be increased by a Valsalva maneuver (which
reduces venous return and LV diastolic volume), by measures to
lower aortic pressure (eg, nitroglycerin)
A mitral regurgitation murmur due to distortion of the mitral
apparatus may be heard at the apex.
When the right ventricular outflow tract is narrowed, a systolic
ejection murmur is sometimes heard in the 2nd interspace at the left
sternal border.
Diagnosis
• Chest x-ray is often done but is usually normal because the
ventricles are not dilated (although the left atrium may be
enlarged).
• ECG shows voltage criteria for LV hypertrophy. Incidence of
preexcitation phenomenon of the Wolff-Parkinson-White
syndrome type is increased. Bundle branch block is common.
• Echocardiography and/or MRI (the best noninvasive confirmatory
tests). Two-dimensional echocardiography can differentiate the
forms of cardiomyopathy and quantify the degree of outflow tract
obstruction, including pressure gradient and area of the stenotic
segment. These measurements are particularly useful for
monitoring the effect of medical or surgical treatment.
• Cardiac catheterization is usually done only when invasive therapy
is considered. Usually, no significant stenoses are present in the
coronary arteries, but elderly patients may have coexisting CAD
Management
• β-Blockers and Ca channel blockers are the mainstays. By
decreasing myocardial contractility, these drugs dilate the heart
and by slowing the heart rate, they prolong the diastolic filling
period. Both effects decrease outflow obstruction, thus improving
ventricular diastolic function
• Avoid drugs that reduce preload (eg, nitrates, diuretics, ACE
inhibitors, angiotensin II receptor blockers) as they increase the
outflow tract gradient and cause a reflex tachycardia that further
worsens ventricular diastolic function.
• Avoid inotropic drugs (eg, digitalis glycosides, catecholamines) as
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they worsen outflow tract obstruction, do not relieve the high

end-diastolic pressure, and may induce arrhythmias.
• Possibly antiarrhythmics (eg, disopyramide, amiodarone)
• Possibly implantable cardioverter-defibrillator and sometimes
surgery or ablative procedures
• Persistent significant symptoms despite medical therapy and
LVOTO peak gradient > 50 mmHg indicate surgical septal
myotomy or myomectomy (Morrow procedure). Mitral valve may
be repaired or replaced if abnormal.
• Dual chamber pacing (DDD) pacemaker may improve symptoms.
• Complications of surgery include Complete heart block (3-5%),
Aortic valve injury, mitral valve injury, and LV pseudoaneurysm
Catheter alcohol ablation is variably effective but is becoming
more widely used.
• Genetic counseling is appropriate for patients with asymmetric
septal hypertrophy.
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Aortic arch obstruction

Definition
Lesions of the aortic arch or proximal portion of the descending aorta
are common and account for
about 5–8% of congenital heart disorders.
Coarctation of the aorta indicates that the aortic lumen is narrowed,
whereas interruption of the aortic arch implies a complete occlusion or
absence of a segment of the aortic arch
Morphology
A) Coarctation of the aorta
• Coarctation of the aorta is either:

a. Diffuse narrowing of arch and isthmus of the aorta has been
referred to as “fetal coarctation” and “tubular hypoplasia of the
aorta.”
b. Localized intraluminal projection of a shelf from the lateral,
posterior, and sometimes anterior wall of the aorta in the
region of the ductus arteriosus. The aortic wall may be
indented at the site of the shelf projection, but the luminal
narrowing is more severe than the apparent indentation.The
location of the shelf is best described as being opposite the
ductus arteriosus termed “juxtaductal coarctation”.
• Collateral circulation between carotid and subclavian arteries

and the descending aorta develops postnatally and begin to
become evident and enlarged depending on the severity of the
coarctation. The vertebral, transcervical, scapular, internal
mammary, and intercostals arteries are all enlarged.
• Coarctation of the aorta is frequently associated with other

cardiac lesions:
a. Mitral valve anomalies, ranging from moderate stenosis to
severe narrowing of the mitral orifice.
b. Subvalvar aortic stenosis may be associated with coarctation of
the aorta as the Shone complex.
c. Ventricular septal defects: most commonly trabecular or
muscular, or perimembranous in location.
d. A bicuspid aortic valve is frequently reported (about 30 to
80%)
• Juxtaductal coarctation of the aorta is commonly associated
with Turner syndrome (about 15% of these individuals).
Aneurysms of the circle of Willis, so-called berry aneurysms,
are thought to occur inabout 10% of individuals with
coarctation.
B) Interruption of the aortic arch
• Complete interruption of the lumen of the aorta is classified,

according to Celoria and Patton, as following:
Ø Type A: interruption between the left subclavian artery and the
ductus arteriosus, i.e., interruption of the isthmus. (20–35% of
cases)
Ø Type B: interruption between the left subclavian and left
common carotid arteries. (60–80% of cases)
Ø Type C: interruption between the left common carotid and the
innominate arteries. (less than 5% of cases).
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• Associated lesions include

Ø Patent ductus arteriosus
Ø Ventricular septal defect: most of these defects are
characterized by maldevelopment of the outflow region with
malalignment of the infundibular septum that creates
narrowing of the left ventricular outflow
Ø Taussig–Bing anomaly, tricuspid atresia with
aortopulmonary transposition and atrioventricular septal
defect with subaortic narrowing. Interruption in this group
of lesions is usually type B.
Ø Truncus arteriosus and with aortopulmonary septal defect.
Aortopulmonary septal defect is usually associated with
type A interruption.
Ø Berry syndrome is a rare combination of interrupted arch,
aortopulmonary septal defect, intact ventricular septum,
and origin of the right pulmonary artery from the ascending
aorta.
Embryological considerations
The exact causes are not known but two mechanisms have been
considered:
(i) Disturbance in embryological development; and
(ii) Disturbance in blood flow patterns.
A) Coarctation of the aorta

(i) Disturbance in embryological development:
Abnormal extension of the ductus arteriosus into the aortic wall and
the constriction of the ductus tissue in the aorta, after birth, results in
coarctation.
Disturbance of migration of the left seventh intersegmental artery that
forms the left subclavian artery.
The decrease in flow across the arch could possibly explain the
development of tubular hypoplasia and even aortic arch interruption.
The site at which the aortic isthmus joins the ductus arteriosus acts as
a branch-point. Normally, the predominant flow pattern is that of
blood passing from the ductus to the descending aorta, so that the
ductus and descending aorta behave as a continuous channel and the
isthmus appears to be a branch entering the side of this channel. If
flow through the ductus arteriosus is increased and that through the
isthmus decreased, the point at the posterolateral junction of the two
arteries will become exaggerated and could thus form the shelf
characteristic of coarctation
B) Interruption of the aortic arch

(ii) Disturbance in embryological development:
Abnormal development of the branchial arterial system
Presence of various degrees of obstruction to outflow into the
ascending aorta, which may restrict blood flow into the aorta and
result in hypoplasia or, if extreme, interruption.
Management
A) Medical therapy
• For symptomatic neonates, prostaglandin E1 infusion(0.01 to
0.1mcg/kg/min—titrate to the lowest effective dose) is required
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for cardiopulmonary stabilization.

• Diuretics can help treat HF symptoms.
• Supplemental O2 should be cautiously used as it may result in
decrease in pulmonary vascular resistance.
• IV cardioactive drugs (eg, milrinone, dopamine, dobutamine)
can be useful in select circumstances (eg, infants with HF and
significant left ventricle dysfunction).
• For hypertension, β-blockers may be used. After repair,
hypertension may persist and can be treated with β-blockers,
ACE inhibitors, angiotensin II receptor blockers, or Ca channel
blockers.
• As DiGeorge syndrome is commonly associated, serum calcium
concentrations should be carefully monitored and hypocalcemia
treated.
B) Balloon angioplasty (sometimes with stent placement):
Initial success rate after balloon angioplasty is about 73% in
patients with native coarctation and about 80% in patients with
recurrent coarctation. Subsequent catheterization can dilate the
stent as children grow.
C) Surgical correction:
• Most prefer surgical correction and reserve the balloon
procedure for recoarctation after surgical correction or for
primary treatment of discrete coarctation in older children or
adolescents.
• Surgical options, for coarctation of aorta, include
Ø Resection and end-to-end anastomosis,
Ø Patch aortoplasty,
Ø Left subclavian flap aortoplasty,
Ø An end-to-side anastomosis of the descending aorta to the
inferior surface of the arch after complete mobilization of the
arch and major branches. Choice of surgical technique depends
on anatomy and center preference
• For interruption of the aortic arch, an end-to-side anastomosis
of the descending aorta to the inferior surface of the arch
proximal to the interruption.
• Endocarditis prophylaxis is not needed preoperatively and is
required only for the first 6 mo after repair
• Aortic arch obstruction with associated defects is repaired as
single (via median sternotomy using deep hypothermia
technique) or staged repair (via left thoracotomy).
• Complications and outcome:
Ø Surgical mortality rate is < 5% for symptomatic infants
and <1% for older children.
Ø Residual coarctation is common (6 to 33%).
Ø Rebound hypetension
Ø Abdominal symptoms (due to mesenteric arteritis),
chylothorax, and recurrent laryngeal nerve injury.
Ø Rarely, paraplegia results from cross-clamping of the aorta
during surgery.
Ø Aneurysm at site of patch repair.
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FIGURE 2−42. Algorithm for the management of IAA.
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Vascular Rings – Slings

Definition
It's malformation of the aortic arch, which may cause compression of
the trachea, oesophagus or both. Most vascular rings result from
either lack of regression of the eighth segment of the right dorsal
aortic root or an abnormal regression of other segments or from
both.
Pathophysiology
A. Double Aortic Arch

• It's the most common type of vascular rings.
• They represent a persistence of both right and left dorsal
aortic arches.
• They arise from the ascending aorta anterior to the trachea,
cross dorsally on both sides of the trachea and oesophagus,
and join posteriorly to form the descending aorta.
• Each arch gives rise to its common carotid and subclavian
arteries, which usually arise as separate branches without an
innominate artery.
• A major posterior arch is more frequently encountered when
the descending aorta is on the left and in most cases of right-
sided upper descending aorta; the right anterior arch is the
major arch.
B. Vascular Compression Associated with a Left-sided Aortic Arch

1. Anomalous Innominate Artery
• In some patients, the innominate artery arises more
posteriorly from the aorta than normally;
• In others, a congenitally short artery may give rise to
tracheal compression.
• In many instances, the anatomy of the great arteries is
normal and the compression is located in the thoracic inlet.
2. Anomalous Left Carotid Artery
This anomaly is similar to the one just described
3. Aberrant Right Subclavian Artery
• It's the most common type of vascular anomaly of the aorta
(approximately 0.5% of the population).
• It results from an embryological interruption of the right fourth

arch between the right common carotid and the right subclavian
arteries.
• In this anomaly, the right subclavian artery arises as the last
branch of the aortic arch and passes obliquely upward from left to
right behind the oesophagus and the trachea to reach the right
arm.
C. Vascular Compression Associated with Left-sided Aortic Arch and

Right-sided Upper Descending Aorta
This is a rare anomaly resulting from interruption of the right aortic
arch between the right subclavian artery and the right ductus
arteriosus. The ascending aorta passes upwards and arches to the
left and around the trachea, proceeds backwards and crosses to the
right behind the oesophagus, and continues to a right-sided upper
descending aorta. The ductus arteriosus may connect the right
pulmonary artery to the right-sided upper descending aorta or to the
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origin of the aberrant right subclavian artery, thus forming a

complete ring around the oesophagus and the trachea.
D. Vascular Compression Associated with Right-sided Aortic Arch

The right-sided aortic arch results from regression of the left dorsal
aortic root. It passes backwards to the right of the trachea and
oesophagus to join the descending aorta. Regression between the
left ductus arteriosus and the descending aorta produces a mirror
image branching of the aorta with the left innominate, right carotid,
and right subclavian arteries arising in that order. Regression of the
left fourth arch between the left common carotid and left subclavian
arteries results in a right aortic arch with aberrant left subclavian
artery.
A left-sided ductus arteriosus may connect the left pulmonary artery
to the upper descending aorta comprising a complete vascular ring
exists.
A right-sided aortic arch, an aberrant left subclavian artery and a
left-sided ductus arteriosus also form a complete ring
E. Cervical Aorta
This is a rare anomaly in which the aorta ascends into the neck on
the right or left side, forming a pulsatile mass in the supraclavicular
region.
F. Pulmonary Artery Sling

In this anomaly, an aberrant left pulmonary artery arises from the
right pulmonary artery, passes over the right main bronchus
posteriorly and to the left, and traverses the mediastinum between
the trachea and the oesophagus to enter the hilus of the left lung.
Hypoplasia of the distal trachea with complete cartilaginous rings is
frequently associated with pulmonary artery sling.
• Present in first 6 months
• Early presentation in double arch
• When symptoms are present, they are caused by compression of
either the trachea or the oesophagus or both.
• Respiratory distress, stridor, cough, repeated chest infections
dating from birth
• Feeding difficulties and dysphagia which tend to become more
apparent when patients begin eating solid foods
• The term ‘‘reflex apnea’’ describes episodes of respiratory arrest
initiated by irritation of the area from tracheal compression
Diagnosis
• Plain chest X-ray and barium oesophagogram are the most
important classic investigations
• The chest X-ray shows the laterality of the aortic arch
• Anteroposterior and lateral oesophagograms show indentations or
compression produced by the vascular anomaly
• Computed tomography (CT) and magnetic resonance imaging
(MRI)- definitive study
• Bronchoscopy may be useful in pulmonary sling
• Two-dimensional echocardiography has a limited value in the
diagnosis of vascular rings, however, remains a very useful tool to
establish the diagnosis of pulmonary artery slings. Also, it's used
preoperatively in all patients to rule out or diagnose associated
intracardiac anomalies.
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Management
A) Double Aortic Arch

Surgical division of the ligamentum, lesser of 2 arches, and any
adhesive bands through left thoracotomy
B) Vascular Compression Associated with a Left-sided Aortic Arch

Anomalous Innominate Artery
• Surgical suspension of the innominate artery from the sternum
through either side with left-sided thoracotomy approach is
better.
Aberrant Right Subclavian Artery
• Surgical division of right subclavian artery through a left-sided
thoracotomy. To avoid subclavian steal syndrome, subclavian
artery may be reimplanted
C) Left Aortic Arch with Right-sided Upper Descending Aorta and

Cervical Aorta
Surgical division of the ductus arteriosus or ligamentum arteriosum
through a right-sided lateral or posterolateral Thoracotomy.
D) Vascular Compression Associated with Right-sided Aortic Arch

Surgical division of the ductus arteriosus or ligamentum arteriosum.
In patients with an anomalous left subclavian artery and
Kommerell’s diverticulum, resection of the diverticulum and
reimplanting the left subclavian artery to the left carotid artery is
recommended. The operation is performed through a left lateral
thoracotomy
E) Pulmonary Artery Sling

The most common procedure is the division and reimplantation of
the left pulmonary artery through a left thoracotomy in the fourth
intercostal space. The left thoracotomy approach remains a
satisfactory option for patients who do not require tracheal and/or
intracardiac surgery. However, another approach can be done
through a median sternotomy.
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FIGURE 2−50.
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Congenital Mitral Valve malformations

Definition
Congenital anomalies of the mitral valve represent a wide spectrum
of lesions that are often associated with other congenital heart
anomalies. They were detected in almost 0.5% of the 13,400
subjects. These lesions can have a variable impact on valve function.
Pathophysiology
A) Morphology
• Carpentier classification of the malformations according to the
motion of the leaflets and the morphology of the papillary
muscles, in one of the three following types:
Ø Type I: Normal leaflet motion. The regurgitation results from
a lack of coaptation between the leaflets.
Ø Type II: Leaflet prolapse. The free edge of one or the two
leaflets overrides the plane of the orifice during systole
Ø Type III: Restricted leaflet motion. The motion of one or the
two leaflets is limited. Two subgroups are described,
depending upon the morphology of the papillary muscles:
Ø Type III1: Restricted leaflet motion, with two clearly visible
papillary muscles.
Ø Type III2: Restricted leaflet motion, with abnormal papillary
muscles.
• An accurate description of the malformations can be achieved

through echocardiography. Thus, the mitral valve should be
analyzed as an entire complex, including the valvar leaflets,
tensor apparatus and papillary muscles.
1. Anomalies of the leaflets
B. Mitral valve prolapse (MVP)

Occurs when the leaflets extend above the plane of the mitral
annulus during ventricular systole.
It is the most common cardiac valvular anomaly in developed
countries.
Myxomatous degeneration is the main aetiology of prolapsing valvar
leaflets, explaining the fact that MVP is uncommon before
adolescence.
When MVP occurs during childhood, it generally integrates into a
congenital disorder affecting the connective tissue, such as Marfan
syndrome, Ehler-Danlos syndrome, osteogenesis imperfecta,
dominant cutis laxa or pseudoxanthoma elasticum. It may be, also,
secondary to a distortion of the left ventricular geometry, as seen in
unrepaired atrial septal defects (right ventricular volume overloading
and left ventricular size reduction). In this case, the mitral valve is
histologically normal and the prolapse usually resolves
postoperatively.
Due to the saddle shape of the mitral valvar annulus, MVP was
broadly overestimated and the normal leaflets can falsely appear to
prolapse in certain echocardiographic views, especially in the apical
two- and four-chamber views.
New echocardiographic criteria have defined MVP as a single or
bileaflet prolapse located at least 2 mm beyond the long-axis
annular plane, with or without a thickening of leaflets.
A classic prolapse is defined as a leaflet thickening exceeding 5 mm,
whereas a prolapse with a lesser degree of leaflet thickening is
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referred to as non-classic.
MVP most commonly involves both leaflets and is symmetrical in
Marfan syndrome, whereas it more frequently affects one leaflet
(posterior) in myxomatous degeneration. The most serious
complication is severe mitral valve regurgitation, although it is
uncommon.
a) Isolated cleft
Isolated cleft of the anterior mitral valve leaflet is a rare but well-
known finding.
It’s a division of one of the leaflets (usually the anterior leaflet) of
the mitral valve.
This must not be mistaken with the so-called ‘cleft’ in AVSD.
It looks like a slit-like hole in the anterior mitral leaflet. Chordal
attachments may connect the edges of the cleft to the ventricular
septum and subsequently create a subaortic obstruction.
More rarely, isolated cleft may be seen in the posterior leaflet of the
mitral valve. Although it may occur at any segment of the posterior
leaflet, the predominant localization of the cleft is within scallop P2.
It results in mitral regurgitation, which is severe in 50% of cases.
C. Double orifice mitral valve (DOMV)

It is a rare condition occurring in 1% of autopsied cases of
congenital heart disease.
DOMV is rarely isolated but usually an ancillary finding in the setting
of a more complex congenital cardiac anomaly. This lesion is usually
found in association with AVSD (52%), obstructive left-sided lesions
(41%) and cyanotic heart disease. Several cases of DOMV were also
reported in association with non-compaction of the left ventricle.
DOMV is defined as a single fibrous annulus with two orifices opening
into the left ventricle.
It differs from duplicate mitral valve, which is defined as two mitral
valve annuli and valves, each with its own set of leaflets,
commissures, chordae and papillary muscles. DOMV must also be
distinguished from an acquired defect after mitral surgery.
DOMV is usually classified into three types:
Ø ‘incomplete bridge type’ is characterized by a small strand of
tissue connecting the anterior and posterior leaflets at the
leaflet edge level;
Ø ‘complete bridge type’, a fibrous bridge divides the
atrioventricular orifice completely from the leaflet edge all the
way through the valve annulus;
Ø ‘hole type’ (eccentric), a secondary orifice with subvalvular
apparatus occurs in the lateral commissure of the mitral
valve.
The two orifices are of equal size in 15% of cases, while a smaller
(accessory) posteromedial orifice is encountered in 44% of cases.
Mitral regurge occurrs in 43% of cases, mitral stenosis in 13% and
both stenosis and insufficiency in 6.5%. There is no functional
consequence of DOMV in 37% of cases.
a) Mitral ring
Mitral ring, also called supravalvar mitral ring or supramitral ring, is
one of the components described in Shone’s syndrome (association
of coarctation of the aorta, subaortic stenosis, PMV and supramitral
ring).
Exceptionally isolated, this lesion is more often associated with
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various other anomalies of the heart, mainly ventricular septal

defects and left-sided obstructive lesions.
According to the relation with the mitral annulus, two types of mitral
rings are described:
Ø The supramitral ring is a fibrous membrane originating just
above the mitral annulus, beneath the orifice of the left atrial
appendage, within the muscular atrial vestibule, not adhering
to the leaflets and associated with a normal subvalvular
apparatus.
Ø The intramitral ring is a thin membrane located within the
funnel created by the leaflets of the mitral valve, closely
adherent to the valve leaflets, always combined with
abnormal subvalvular apparatus.
The supramitral ring must be distinguished from cor triatriatum
sinister, which is a fibromuscular membrane, clearly separated from
the mitral valve (proximal to the left atrial appendage) that divides
the left atrium into two parts.
Supramitral ring may be described as a valvar lesion rather than
supravalvar because the annulus is an integral part of the mitral
valve. The ring can be either complete, circumferential or partial.
It creates a stenosis that is usually progressive with a median age at
diagnosis of 36 months.
b) Ebstein’s malformation of the mitral valve

Ebstein’s malformation of the left-sided atrioventricular valve has
been reported a few times in cases of corrected transposition of the
great arteries, but, in this situation, the involved valve was obviously
of tricuspid morphology.
The malformation exclusively affects the posterior valve leaflet,
which is plastered into the left ventricle wall, thus displacing the
mitral valve orifice downward into the left ventricle.
Unlike Ebstein’s malformation of the tricuspid valve, the atrialized
inlet portion is usually not thinned.
This exceedingly rare anatomical condition causes mitral
insufficiency
4. Anomalies of the tensor apparatus
a) Arcade or hammock valve

It’s a direct connection of the papillary muscles to the mitral leaflets,
either directly or through the interposition of unusually short
chordae. It’s called hammock valve because it mimics a hammock
when the valve is observed from an atrial aspect (as seen by a
surgeon).
The tendinous cords are thickened and extremely short, thus leading
to an abnormal excursion of the leaflets that may cause both
stenosis and insufficiency.
The space between the abnormal chordae may be completely
obliterated, and in the most severe form, the papillary muscles are
directly fused with the free edge of the leaflet.
b) Straddling mitral valve (SMV)

It is defined by an abnormal attachment of the mitral chordae to
both ventricles.
SMV is consequently always associated with a ventricular septal
defect.
The mitral valve always straddles through a conoventricular
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(misalignment) type of ventricular septal defect.

SMV is almost always associated with conotruncal anomalies, such
as double outlet right ventricle or transposition of the great arteries.
SMV must be distinguished from the overriding of the mitral valve,
which qualifies a mitral annulus committed to the two ventricular
chambers.
5. Anomalies of the papillary muscles
a) Parachute mitral valve (PMV)

Among the causes of congenital mitral stenosis, PMV is frequently
encountered, as shown by the incidence of 0.17%.
True PMV is characterized by unifocal attachment of the mitral valve
chordae to a single (or fused) papillary muscle. This single papillary
muscle is usually centrally placed and receives all chordae from both
mitral valve leaflets.
Although the spectrum of associated lesions is broad, PMV is
commonly seen in association with other obstructive lesions affecting
the left heart or conotruncal anomalies.
Because opening of the mitral valve is limited, true PMV is highly
associated with mitral stenosis. Mitral regurgitation occurs less
commonly but must be equally carefully followed because of its
progressive evolution.
B) Hemodynamic consequences:
Either mitral stenosis or mitral regurgitation results in progressive
pulmonary venous hypertension, however, regurgitation is tolerated
slightly better with LV volume overload and eventually HF.
The clinical presentation is variable, mainly depending on the
associated cardiac lesion. Symptoms are related to the degree of
mitral insufficiency and/or stenosis
Diagnosis
• ECG: LA enlargement and RV hypertrophy.

• CXR: enlarged LA, increased pulmonary vascular markings.
• Echo: this is diagnostic and some time and effort should be spent
in analysing the mechanism of valve dysfunction. Associated
lesions must be sought
Management Selected Articles

a) Medical: Rodefeld MD, Brown JW,
• Diuretics are given in the context of mitral stenosis and Heimansohn DA, King H, Girod DA,
Hurwitz RA, Caldwell RL. Cor
vasodilators and transfusion in the presence of mitral
triatriatum: clinical presentation
regurgitation
and surgical results in 12 patients.
• Vasodilator therapy is not recommended for the treatment of Annals of Thoracic Surgery 1990
asymptomatic patients with severe mitral regurgitation and 50(4):562-8.
normal left ventricular function, as this may increase the risk of Salomone G, Tiraboschi R, Bianchi
paradoxical worsening in mitral regurgitation T, Ferri F, Crippa M, Parenzan L.
Cor triatriatum. Clinical
b) Surgical: presentation and operative results.
• In MVP: Mitral valve repair is recommended in patients with Journal of Thoracic and
Cardiovascular Surgery 1991
symptomatic severe mitral regurgitation or in asymptomatic
101(6):1088-92.
patients with ventricular enlargement or dysfunction. Surgical
Sources for further reading
technique consists of resection of the prolapsed part of the leaflet, Textbook Chapters
25
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72
with or without an annuloplasty Chapter 36: Cor Triatriatum

• Mitral valve repair of isolated cleft associated with mitral Sinister, Pulmonary Vein Stenosis,
regurgitation is preferred to mitral valve replacement and usually Atresia of the Common Pulmonary
Vein, and Cor Triatriatum Dexter.
consists of a direct suture of the cleft
Pediatric Cardiac Surgery
• In the absence of an associated lesion requiring surgery, repair of
(Mavroudis and Backer), 2nd ed.,
DOMV is usually not necessary. When DOMV is associated with 485-504.
potentially PMV and AVSD, the cleft that represents the larger Chapter 17: Cor Triatriatum.
orifice of DOMV should not be closed completely to avoid severe Cardiac Surgery (Kirklin and
iatrogenic MS. In such a case, mitral valve replacement is Barratt-Boyes), 2nd ed., 645-682.
sometimes helpful
• For supramitral ring, ring excision can be achieved with no need
for reoperation, compared with frequent recurrence (50%) in case
of intramitral ring. In such cases, concomitant surgery of the
tensor apparatus must often be performed to obtain sufficient
haemodynamics.
• When necessary, for hammock valve, conservative surgery will
create two separated papillary muscles by resection of the
muscular band
• Surgical management of SMV is closely dependent on the more
complex associated cardiac anomaly.
• For PMV, conservative surgical treatment may consist of either
chordal fenestration or papillary muscle splitting, associated or
not with a commissurotomy. Univentricular palliation may be
needed in in cases of Shone’s syndrome with left ventricular
hypoplasia
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FIGURE 2−59. Algorithm for the management of MS. Bonow RO, Caranetto, BA, Chaterjee K, et al.
Circulation 2006;114;e84.
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03 04
Acyanotic Cyanotic
w L®RS PBF
02 05
Acyanotic Cyanotic
w/o Shunt ¯PBF
R. Side
01 06
Acyanotic Cyanotic
w/o Shunt PAH
L. Side
ezzeldinmostafa.com
74
Dr. Ezzeldin Mostafa emostafa@med.asu.edu.eg 3. Cyanotic w/o Shunt, R-sided Lesions
3 Acyanotic, without Shunt, R-sided Lesions

Table of Contents
Supravalvular Pulmonic (Pulmonary) Stenosis ..... 8
3 Acyanotic, without Shunt, R-sided Ebstein's Anomaly ................................................. 8
Lesions ...........................................................2
RVOTO .................................................................. 4
Pulmonic (Pulmonary) Stenosis ............................ 5
2
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(CHD)
terminology
om/pedscards-
Timeline
RVOTO
• RVOTO is usually congenital, but symptoms usually do not appear
until adulthood.
• Pulmonary stenosis and pulmonary atresia with intact ventricular
septum are classically described as two separate entities.
However, there is a consensus that in the neonatal period, critical
pulmonary stenosis and pulmonary atresia often have a similar
clinical presentation, and exhibit the same anatomical features of
right ventricular (RV) hypertrophy and cavity hypoplasia.
• Heart sounds include increased splitting of S2 and a harsh
crescendo-decrescendo ejection murmur heard best at the left
parasternal 2nd or 4th intercostal space.
• Management is indicated according to symptoms and severity of
obstruction.
• Principles of management include providing pulmonary forward
flow, stimulate right ventricular growth, enable the right ventricle
to serve the pulmonary circulation, and right ventricular
decompression to avoid left ventricular compromise and to be
incorporated later into a biventricular circulation.However, the
right ventricle should not be decompressed in the presence of a
right ventricular-dependent coronary circulation
• The decision between univentricular and biventricular repair
depends on the size of the right ventricular cavity and the
presence or absence of coronary artery fistulae
• The options available include the following:
1. Maintaining ductus arteriosus patency
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2. Transcatheter opening of the pulmonary valve

3. Surgical opening of the pulmonary valve and, if necessary, the
right ventricular outflow tract.
4. Introducing a systemic arterial to pulmonary arterial shunt
surgically.
5. Introducing a systemic venous to pulmonary arterial shunt.
6. Closing the tricuspid valve.
Ebestein anomaly
Ebestein anomaly has the following features:
1. Adherence of the tricuspid valve leaflets to the underlying
myocardium (failure of delamination)
2. Apical displacement of the septal and posterior leaflets of the
tricuspid valve below the AV junction in the right ventricle
3. Atrialization and dilation of the inflow of the right ventricle to
varying degrees
4. Redundancy, tethering, and fenestrations of the anterior
tricuspid valve leaflet
5. Varying degrees of TR
6. Enlargement of the right atrium
7. Associated lesions include either a PFO or secundum ASD, One
or more accessory conduction pathways, WPW, and
occasionally, other anomalies such as mitral valve prolapsed
and Abnormalities of LV morphology and unction
• Patients experience varying degrees of cyanosis,
cardiomegaly, congestive heart failure, exercise intolerance
and symptomatic arrhythmias.
• The primary operation generally consists of closure of any
interatrial communications; antiarrhythmia procedures such as
surgical division of accessory conduction pathways,
cryoablation of AV node reentry tachycardia, or Maze
procedure; and tricuspid valve surgery. A right reduction
atrioplasty is often performed.
• The tricuspid valve is repaired when feasible, and tricuspid
valve replacement is performed with a mechanical or
heterograft bioprosthesis when repair is not feasible or the
repair result is not satisfactory.
• A bidirectional cavopulmonary anastomosis is considered in
selected patients with severe RV dysfunction and preserved LV
function with low left atrial pressure.
• The single-ventricular-Fontan pathway may be considered for
profound RV dysfunction most often when operation is
required during infancy.
•
RVOTO
Pathophysiology
Obstruction to RVOT can be in various forms:
A) Congenital
1. Valvular stenosis
• Dome-shaped pulmonary valve
• Dysplastic pulmonary valve
• Unicuspid or bicuspid pulmonary valve
• May be associated with Noonan syndrome and carcinoid syndrome
in adults
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1. Infundibular stenosis
• Usually associated with tetralogy of Fallot
• May be associated with pulmonary valve stenosis (reactive
myocardial hypertrophy)or hypertrophic cardiomyopathy
• Causes other than muscular like tricuspid valve tissue, aneurysm
of the sinus of valsalve, and aneurysm of the membranous septum
2. Subinfundibular obstruction
• Double-chambered right ventricle
2. Supravalvular stenosis
• Hourglass deformity at valve
• Pulmonary artery membrane
• Pulmonary artery stenosis
• Peripheral pulmonary artery stenosis
• Usually associated with rubella, Williams, and Keutel syndromes.
3. Pulmonary atresia with intact ventricular septum
B) Postoperative
• Native valve restenosis
• Prosthetic valve stenosis
• Post pulmonary artery banding
• Conduit stenosis
• Peripheral stenosis after arterial shunts procedure to pulmonary
arteries.
Diagnosis
1. Chest x-ray shows:
• Normal heart size unless there is an associated cardiac lesion.
• Chen's sign: vascular fullness in the left lung base greater than the
right base due to the preferential pulmonary flow to the left lung in
patients with PS.
• Dilatation of the main pulmonary artery is common in doming PS.
• Calcification of the valve may rarely be seen in older patients.
• The right atrium may be enlarged.
2. ECG may be normal or show RV hypertrophy or right bundle
branch block
3. Echocardiography confirms the diagnosis and can characterize the
severity.
4. Magnetic Resonance Imaging/Computed Tomography is
• Confirmatory
• Diagnosis of stenosis of the main, branch, and peripheral
pulmonary arteries
• Useful when these associated lesions are of concern
• To assess the degree of pulmonary regurgitation or TR
5. Right heart catheterization is indicated only when
• 2 levels of obstruction are suspected (valvular and infundibular),
• when clinical and echocardiographic findings differ,
• or before intervention is done.
Pulmonic (Pulmonary) Stenosis

Pathophysiology
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A) Classification of severity:
• Most classifications of severity have been based on the pressure
gradient across the RVOT:
• Pressure gradients below about 40 mmHg have been
considered to represent mild stenosis,
• Gradients of 40–80 mmHg represent moderate stenosis,
• Gradients above 80 mmHg represent severe stenosis.
• Similarly, degree of severity has been based on the relation
between right ventricular systolic pressure and systemic systolic
pressure.
• The stenosis has been considered mild when right ventricular
systolic pressure is less than half systemic systolic pressure,
• Moderate when it is above half but less than systemic,
• Severe when it is greater than systemic systolic pressure.
Drawbacks of these classifications:
Ø They may not be appropriate in neonates and infants, because
the high right ventricular pressures noted with severe stenosis
in older infants and children may not be developed yet.
Ø Presence of significant tricuspid regurgitation or reduced
cardiac output may limit the levels of right ventricular systolic
pressures achieved, even with severe stenosis.
Ø The gradient may be markedly altered by changes in heart
rate and systolic ejection time.
• A more reliable classification is based on valve orifice area in

patients with valvar stenosis. Normally, the pulmonary valve
orifice diameter is about 2.0 cm/m2 body surface area and
pulmonary valve area is about 2.5– 3.0 cm2/m2. Experimentally,
the pulmonary artery has to be constricted to less than one-third
its diameter to produce a significant pressure gradient.
• Mild pulmonary stenosis can be considered to be present when
the valve area is 0.75–1.0 cm2/m2 and valve diameter is 8–
10 mm/m2.
• Stenosis is severe when pulmonary valve area is less than 0.3
cm2/m2 or valve diameter is less than 3 mm/m2.
• Values between these ranges are considered to represent
moderate stenosis
B) Associated lesions:
• Many other forms of complex CHD
• Beyond infancy, poststenotic dilatation of the main and
proximal left pulmonary arteries is a common finding in
patients with pulmonary stenosis
• RV cavity :
Ø The obstruction produces concentric hypertrophy of the right
ventricle, which may result in secondary infundibular stenosis
with subsequent mild to moderate reduction in right
ventricular cavity size.
Ø Severe right ventricular hypoplasia or enlargement is rare.
The onset of symptoms in patients with pulmonary stenosis is related
to the severity of the obstruction.
a) Severe PS
• May present with serious difficulties within 24–48 hours after
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birth
• Cyanosis may be present at birth, but even with pulmonary
atresia it may be mild, because pulmonary blood flow is
provided by the ductus arteriosus. Cyanosis may increase
progressively during the first 7–10 days with closure of the
ductus arteriosus.
• Severe cyanosis is associated with tachypnea, mottling and
metabolic acidosis.
• In the presence of tricuspid insufficiency, the cardiac impulse
is hyperactive over the whole precordium.
• In infants with severe pulmonary stenosis, the second sound
in the pulmonary area is of low intensity but may be widely
split. A systolic ejection murmur of variable duration may be
heard at the upper left sternal border.
b) Mild to moderate PS
• Many children remain asymptomatic for years and do not
present to a physician until adulthood.
• Cyanosis on exertion may be present if foramen ovale is still
open.
• A prominent jugular venous a-wave (due to forceful atrial
contraction against a hypertrophied RV), an RV precordial lift
or heave, and a left parasternal systolic thrill at the 2nd
intercostal space.
On auscultation, the 1st heart sound (S1) is normal and the splitting
of the 2nd heart sound (S2) is widened (pulmonic component of S2, is
delayed). A systolic ejection click is usually heard along the left
sterna border. A harsh crescendo-decrescendo ejection murmur is
audible and is heard best at the left parasternal 2nd (valvular
stenosis) or 4th (infundibular stenosis) intercostal space with the
diaphragm of the stethoscope when the patient leans forward
Management
A) Symptomatic neonate with critical PS
1. Resuscitative measures:
• Keeping normothermia to minimize O2 consumption
• PGE1 infusion (0.05-0.1µg /kg /min) to maintain PDA and
improve O2 saturation.
• Oxygen should be administered
• Correction of acidosis and hypoglycemia
• Transcatheter PDA stenting
2. For patients with small right ventricles, whose cavity is made of an
inlet portion only, a modified Blalock–Taussig shunt is procedure of
choice.
3. In patients with a patent infundibulum, relief of right ventricular
outflow obstruction can be accomplished using a variety of
procedures:
• Percutaneous pulmonary balloon valvuloplasty.
• Surgical valvotomy which can be done with or without
cardiopulmonary bypass
• Surgical transannular patch with or without surgical resection
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of the valve.
B) Infants and children

• Symptomatic patients and a systolic pressure gradient across
the pulmonary valve of greater than 40-50 mmHg at rest were
considered an indication for intervention.
• Procedures:
Ø Percutaneous pulmonary balloon valvuloplasty.
Ø Surgical valvotomy/valvectomy which can be done with or
without cardiopulmonary bypass
Ø Surgical infundibular resection (in secondary infundibular
hypertrophy) with or without transannular patch.
Supravalvular Pulmonic (Pulmonary) Stenosis

•The clinical symptoms are similar to that of valvular PS.
•Findings of certain associated syndromes like congenital
Rubella syndrome.
Management
• Focal branch and/or peripheral pulmonary artery stenosis
with greater than 50% diameter narrowing, an elevated RV
systolic pressure greater than 50 mm Hg, and/or symptoms
indicate intervention.
• Percutaneous interventional therapy is recommended as the
treatment of choice.
• The highly elastic pulmonary arteries have proved resilient to
balloon procedures, and angioplasty methods have generally
given way to stent procedures in which there appears to be a
higher initial success rate and a lower intermediate-term
incidence of restenosis
Ebstein's Anomaly
Definition
Ebstein's anomaly is a rare congenital malformation that accounts for
approximately 1% of all congenital defects. It encompasses a wide
spectrum of anatomic and functional abnormalities of the
morphological tricuspid valve and right ventricle
Pathophysiology
A) Morphology
· The septal and posterior leaflets of the tricuspic valve are displaced
inferiorly towards the RV apex
· The anterior leaflet is large and sail-like with abnormally numbered
and placed chordal attachments
· The area of right ventricle between the true tricuspid annulus and the
displaced attachment of the septal and posterior leaflets is thinned and
dilated (atrialized)
· The remainder of the right ventricular cavity is small
· The valve leaflets may be adherent to the right ventricular wall
· The RV infundibulum can be obstructed by the anterior leaflet and/or
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its chordal attachments

· The conduction system follows its normal course
· The anomaly can be right or left-sided
· Left-sided lesions are usually in the setting of corrected TGA and
have a morphologic right-sided valve
B) Carpentier's classification
Type a:
The anomaly is limited to the displacement of the septal leaflet with a
small atrialized chamber.
The anterior leaflet is large, moves freely and has a free leading edge
between the anteroseptal and anterolateral commissures.
Type b:
The atrialized chamber is large
The anterior leaflet is restricted, with some interchordal space
obliteration and attachment of the ventricular surface to the ventricle
by fibrous bands.
The posterolateral and anteroseptal commissures are clearly
delineated
Type c:
There is a marked displacement of the posterior and septal leaflets,
which may be severely hypoplastic
The atrialized chamber is large and has a thin, non-contractile wall.
The functional right ventricle is small and its contractility may be
diminished.
The anterior leaflet is partially fused to the infundibulum and the
trabecular portion of the right ventricle. The ventricular edge of the
valve is adherent to the ventricle,
Type d: (Uhl's syndrome)
The right ventricle is entirely made of the atrialized chamber
No anterior or posterior leaflets can be recognized, as they are totally
adherent to the ventricular wall.
The tricuspid orifice is deeply displaced towards the infundibulum and
is often stenotic.
C) Associated cardiac anomalies

· PFO or ASD most common
· Right ventricular outflow tract obstruction
· Wolfe-Parkinson-White syndrome
Clinical Presentation
A. Contributing factors
· Severity of tricuspid incompetence
· Presence of associated atrial septal defect
· Right ventricle impairment
· Other cardiac anomalies
· There is a broad range of symptom severity and age of presentation
B. Neonatal Presentation
· Worst deformity of valve and RV
. Varying degrees o cyanosis according to patency of ductus arteriosus
· Tricuspid incompetence accentuated by normal elevated pulmonary
resistance
· Associated pulmonary stenosis or atresia
· Pulmonary hypoplasia correlated with tricuspid valve incompetence
C. Other Features
· Right ventricular dysfunction
· Cardiomegaly, hepatomegaly, ascites, fluid retention
· Tricuspid valve incompetence
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· Cyanosis
· Atrial level R to L shunt, polycythemia
· Paroxysmal tachycardia
· Wolff-Parkinson-White Syndrome
· Paradoxical embolus
· Cerebral abscess
D. Natural History
· Prognosis is poor with presentation during the first week of life
· Prognosis improved with presentation after infancy and with mild
symptoms
· Exercise tolerance often reduced
· CHF, sudden death, and paradoxical emboli are the three most
common causes of death
Diagnosis
· Chest X-ray characteristically shows a globular heart from the
enlarged right atrium
· 2-D echocardiography is diagnostic and accurately evaluates the
following:
a) RA size
b) tricuspid annulus size
c) function of the valve leaflets
d) evidence of RVOTO
e) presence of PFO or ASD
f) degree of valve incompetence
Management
A) Medical Danielson GK, Driscoll DJ, Mair
· PGE for the neonate DD, Warnes CA, Oliver WC Jr.
· General supportive care of cyanotic infants Operative treatment of Ebstein's
· All patients eventually show progressive deterioration and will anomaly. Journal of Thoracic and
become possible candidates for surgery Cardiovascular Surgery 1992
104(5):1195-202.
A large series of 189 patients
B) Surgical
with a mean follow-up of 16 years.
Indications for Operation
Mortality was low, reoperative rate
· Significant tricuspid valve incompetence was low (4%) in patients undergoing
· Moderate to severe cyanosis with compensatory polycythemia tricuspid valve reconstruction, and
· Congestive heart failure (NYHA class III or IV) the vast majority of patients have
· Extreme cardiomegaly excellent functional status.
· Arrhythmias Gentles TL, Calder AL, Clarkson
PM, Neutze JN. Predictors of long-
Operative Technique term survival with Ebstein's anomaly
· Tricuspid valve repair is preferred of the tricuspid valve. American
· Replace the valve if unable to repair Journal of Cardiology 1992
· Plicate the atrialized right ventricle 69(4):377-81.
· Close any associated ASD An elevated cardiothoracic ratio,
· Interrupt accessory conduction pathways if present poor NYHA class, and the
. Bidirectional Glenn shunt in patients with a large functional right development of atrial fibrillation
ventricle of poor systolic function. were some of the predictors of death
in this series.
Operative Modifications Carpentier A, Chauvaud S, Mace
L, Relland J, Mihaileanu S, Marino
A. Repair with tricuspid valve replacement
JP, Abry B, Guibourt P. A new
· Atrial plication
reconstructive operation for Ebstein's
· The original operation replaced the tricuspid valve with a prosthetic anomaly of the tricuspid valve.
ball valve Journal of Thoracic and
· Current valve replacement is usually done with a mechanical Cardiovascular Surgery 1988
prosthetic valve 96(1):92-101.
B. The Mayo (Danielson) annuloplasty This method of preserving the
· Pledgets placed in ventricle and woven up to level of annulus tricuspid valve with repositioning
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· Sutures tightened to eliminate atrialized portion and form a results in improved NYHA functional
competent valve class, improvement in arrhythmias,
C. Ring annuloplasty and good valve function on echo.
· Remodels the shape of the annulus to improve valve competence
D. The Carpentier repair
· Mobilize behind the anterior leaflet and repair any fenestrations or
perforations
· Vertically plicate of the atrialized portion
· This approach has less tension and reduces the diameter of the
tricuspid annulus
· Advance the anterior leaflet across the plicated area to cover the
orifice
· Placement of an annular ring supports the repair
E. The Quaegebeur repair
· Similar to Carpentier repair; annuloplasty ring is omitted
F. The Starnes operation
· Neonates in extremis may require urgent intervention
· The ASD is enlarged by excising all of the septum primum
· The tricuspid valve is closed with a pericardial patch
· The right atrium is plicated
· A systemic-pulmonary shunt is created
· If right ventricular function is inadequate, a Fontan operation may be
necessary
Results Sources for further reading
· Early (hospital) mortality is about 5% Textbook Chapters
· Acute heart failure is the principal cause of early mortality Chapter 28: Ebstein's Anomaly.
· Late death is uncommon Pediatric Cardiac Surgery
· The tricuspid valve is usually competent or has mild incompetence (Mavroudis and Backer), 2nd ed.,
long-term, with a low rate of reoperation (3-5%) 413-24.
· Heart block is uncommon Chapter 16: Ebstein's Anomaly.
Cardiac Surgery of the Neonate and
· W-P-W cured with surgical interruption
Infant (Castaneda, Jonas, Mayer and
· NYHA class is I or II in the majority of patients
Hanley), 273-80.
· Exercise tolerance and oxygen uptake are improved on maximal
Chapter 27: Ebstein's Malformation.
exercise testing Cardiac Surgery (Kirklin and
Barratt-Boyes), 2nd ed., 1105-30.
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Ebstein’s Anomoly
Epi
-Tri Vlv xx, <1% CHD
Path
-septal & post leaflet is
displaced downwardàtop of RV is in RA (“atrialized RV”)à Fx’l RV
hypoplasia.
-TR
-+/- (rare) redundant Tri vlv tissue à RVOTà RV dilation/hypertrophy
-ASD/PFO RàL in all pts to allow flow from RA to LV to Ao to PDA to PA to lungs
-thin, dilated RV free wall, fibrosis at both RV & LV free wallà severe Sx early, then LV dysfx
later
-WPW preexcitation assoc’d à SVT
-PS, Pulm atresia, TOF, VSD associated sometimes
Hx
-cyanosis & CHF at WOL#1 if severe, but might improve as
PVR
-if mildà cyanosis, fatigue, SOB, palpitation w exertion
-+/- SVT Hx
PE
-cyanosis, clubbing (if older)
EKG- RBBB, RAH in most pts; 1st AV block (40%).
-triple/quadruple rhythm- widely
WPW in 15-20% +/- SVT
spit S2. Split S1, S3, S4. Soft
holosystolic/early systolic
CXR- Nl if mild, severeà extreme cardiomeg at RA,
murmur of TR at LLSB. Soft,
balloon shaped heart w Pulm vssls
scratchy, mid-diastolic murmur
-hepatosplenomegaly
Echo-
NHx -apical displacement of the hinge pt of the septal leaflet
-20% die as newborns if +Sx, of tri valve. Nl = septal leaflet insert on septum at just
30% more diet by 10yo, from below MV, but here it is much lower. Dx= >8mm/m2
CHF. Avg die at 20 yrs. toward apex. See it in 4 chamber view.
- PVR as newbornà improve -elongated, redundant, dysplastic tri valve leaflets w
cyanosis as it is easier for RV to abnormal chordal attachment
pump Q to lungs -big RA, small fxl RV. TR & TS
-if less severe anomalyà less -+/- RVOT obst due to redundant ant leaflet
Sx/ASx -nonrestrictive ASD
-Cyanosis, CHF, LV dysfx fortell -+/- MVP, LV dysfx
early death
-SVT w WPW in 15-20%, +/-
sudden unexpected death bc
arrhythmia
-Other xx= IE, brain abscess,
CVA
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85

RxTx
-severely cyanotic newbornsà mech SurgTx
vent, PGE1, inotropes, correct -Indications: critically ill at WOL#1, mod/sev
metabolic acidosis, then c/s emergent cyanosis w <80%sat, polycythemia (Hgb16),
surgery CHF; RVOT bc redundant tissue, Class III-IV
-mild infantà try above & if improve Tx activity limitation, h/o paradoxical embolus, life-
w PGE1/inotropes w gradual w/drawal, threatening arrhythmias.
watch closely as PDA closes -Palliative Procedures:
-ASx kids w mild Ebsteins- observe -BT shunt w enlargement of ASD, then
only, then diuretics/digoxin if CHF Fontan
-Acute SVT- adenosine; beta blocker to -Definitive Procedures
prevent SVT; c/s ablation if recurrent -if good RV size & fx, then fix both
SVT bc reentry ventricles; if ng then need Fontan
-SBE ppx prn -Reconstruct TriVlv or replace it.
-activity restriction prn
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FIGURE 3−7. Initial medical management algorithm and management algorithm of Ebstein’s patients
based on associated anomalies and overall stability. Knott-Craig C J et al. World Journal for Pediatric and
Congenital Heart Surgery 2012;3:16-20.
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03 04
Acyanotic Cyanotic
w L®RS PBF
02 05
Acyanotic Cyanotic
w/o Shunt ¯PBF
R. Side
01 06
Acyanotic Cyanotic
w/o Shunt PAH
L. Side
ezzeldinmostafa.com
88
Fundamentals Spring 2016
Dr. Ezzeldin Mostafa emostafa@med.asu.edu.eg Acyanotic w Shunt
4 Acyanotic with shunt

Table of Contents Congenital Coronary Artery Anomalies &
Coronary artery fistula ......................................... 18
4 Acyanotic with shunt ................................ 2
Congenital Arteriovenous Fistulas ....................... 19
Atrial septal defects & Partial anomalous
Anomalous Origin of Left Coronary Artery from
venous connection ................................................ 7
the Pulmonary Artery (ALCAPA) ......................... 20
Sinus Venousus ASD: ..................................... 10
Congenital anomalies of Sinus of Valsalva .......... 20
Ostium Primum ASD: ...................................... 10
Patent Ductus Arteriousus (PDA) ........................ 22
Partial anomalous pulmonary venous
Aortopulmonary Septal Defect (AP Window ) ...... 24
connection (PAPVC) ........................................... 11
Atrioventricular Septal Defect (AVSD) ................. 25
Ventricular Septal Defect (VSD).......................... 14
2
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(CHD)
terminology
om/pedscards-
Timeline
ASDs
• The hemodynamic changes and clinical manifestations of the
PAPVC and atrial septal defects are almost identical as they
depends on left-to-right shunting.
• Small atrial communications often close spontaneously, but larger
ones do not, causing right atrial and ventricular overload and
ultimately pulmonary artery hypertension, elevated pulmonary
vascular resistance, and right ventricular hypertrophy; SVT, atrial
flutter, or atrial fibrillation may also occur.
• ASDs can allow emboli from the veins to enter the systemic
circulation (paradoxical embolization), causing arterial occlusion
(eg, stroke).
• Auscultation typically reveals a grade 2 to 3/6 midsystolic murmur
and a widely split, fixed S2; these findings may be absent in
infants.
• Moderate to large ASDs should be closed, typically between ages 2
yr and 6 yr, using a transcatheter device when possible.
• Sinus venosus and ostium primum defects and PAPVC are not
amenable to device closure.
• Patent foramen ovale has been detected in 44–66% of cryptogenic
stroke (i.e.unknown cause) patients, compared with 9–27% in
patients with a known cause of stroke. The presence of PFO
increases the incidence of recurrent events fivefold despite
treatment with anticoagulant or antiplatelet drugs. Closure of the
PFO by surgery or device significantly reduced the risk of recurrent
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90
stroke, so, it is generally agreed that if recurrent episodes of

stroke have occurred despite the use of warfarin prophylaxis, the
PFO should be closed by a device. However, its closure to prevent
possible occurrence of stroke is not recommended
• 54% of patients with migraine and aura had a patent foramen
ovale. 70% of individuals who had closure of the PFO after a
cryptogenic stroke found that migraine they had experienced prior
to the stroke disappeared; the other 30% of individuals showed
considerable improvement. Although it is possible that closure of
PFO may be indicated for treatment of severe migraine with aura,
the effectiveness of the procedure requires more confirmation.
VSDs
• VSD is an opening in the interventricular septum, causing a left-to-
right shunt.
• Over time, large left-to-right shunts cause pulmonary artery
hypertension, elevated pulmonary artery vascular resistance, right
ventricular pressure overload, and right ventricular hypertrophy,
which ultimately cause shunt direction to reverse, leading to
Eisenmenger syndrome.
• Large defects cause dyspnea with feeding, poor growth during
infancy, recurrent respiratory infections and symptoms of heart
failure (HF) at age 4 to 6 wk.
• Typically, a grade 3 to 4/6 holosystolic murmur at the lower left
sternal border is audible shortly after birth.
• Diagnosis is by echocardiography
• Defects may close spontaneously during infancy or require surgical
repair.
• Infants who do not respond to medical treatment of HF or have
poor growth should have surgical repair during the first few
months of life; even asymptomatic children with large defects
should have repair during the first year of life.
Coronary Anomalies
• Coronary anomalies may be congenital or acquired
• Loss of coronary perfusion may occur due to causes such as
anatomic torsion, extrinsic compression, focal or diffuse
fibrocellular intimal thickening, small-caliber distal coronary
arteries, coronary aneurysms or fistulas, and ectopic origins of the
coronary arteries with functional decrease in coronary flow reserve
• Noninvasive ischemia provocation testing for myocardial ischemia
is recommended if possible. If further definition of coronary artery
anatomy were suggested, other imaging modalities such as cardiac
catheterization, intravascular ultrasonography, CT angiography, or
magnetic resonance angiography are useful.
• Management can be surgical or catheter-based revascularizations.
Abnormalities of the sinus of Valsalva
• Abnormalities of the sinus of Valsalva may be congenital or
acquired.
• It may rupture into one of the adjacent lowered-pressure cardiac
chambers.
• Surgical resection of the aneurysm and obliteration of the fistula
tract is the management.
PDA
• Patent ductus arteriosus (PDA) is persistence after birth, beyond
the age of three months, of the normal fetal connection (ductus
arteriosus) between the aorta and pulmonary artery, resulting in a
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left-to-right shunt.
• Manifestations depend on the size of the PDA and the degree of
prematurity, but a continuous murmur is characteristic and, if
loud, has a “machinery-sounding” quality. Diagnosis is by
echocardiography
• Premature infants may have respiratory distress or other serious
complications (eg, necrotizing enterocolitis).
• Over time, a large shunt causes left heart enlargement, pulmonary
artery hypertension, and elevated pulmonary vascular resistance,
ultimately leading to Eisenmenger syndrome.
• Administration of prostaglandin inhibitors with or without fluid
restriction may be tried in premature infants with a significant
shunt, but this therapy is not effective in term infants or older
children with PDA. surgical closure has not been shown to improve
outcomes.
• If the connection persists, surgical or catheter-based correction is
indicated.
APW
• AP window is a defect of conotruncal development in which there is
incomplete separation of the aorta and pulmonary arteries
• It results in a high flow left to right resulting in increased
pulmonary blood flow and LV volume overload and failure with
early development of Eisenmenger syndrome and survival after 1
year is rare if untreated.
• Presentation is much like a PDA and depends on size of the defect,
the PVR, and other associated malformations. Failure to thrive and
frequent respiratory infections are common.
• Diagnostic study of choice is echocardiography; cardiac
catheterization also helpful to evaluate associated abnormalities
Infants in heart failure should be treated medically followed by early
surgical closure of the defect.
AVSDs
• AVSDs may be complete, transitional, or partial; the majority
of patients with the complete form have Down syndrome.
• AVSD involves a large (ASD/OP)+VSD +common AV valve
(often with significant regurgitation), all resulting in a large
LèRS at both atrial and ventricular levels and enlargement of
all 4 cardiac chambers.
• A partial AVSD also involves an ASD, but the common AV
valve is partitioned into 2 separate AV orifices and there is no
VSD, resulting in enlargement of the right heart chambers
because of a large atrial shunt but no ventricular shunt.
• A transitional AVSD involves an ostium primum ASD, a
common AV valve, and a small- or moderate-size VSD.
• Complete AVSD with a large LèRS causes signs of CHF by age
4 to 6 wk.
• Symptoms in partial AVSD vary with the degree of MR; if mild
or absent, symptoms may develop during adolescence or early
adulthood, but infants with moderate or severe MR often have
manifestations of CHF.
• Symptoms in transitional AVSD fall on a spectrum, depending
on the size of the VSD.
• Defects are repaired surgically between age 2 to 4 mo and 1
to 3 yr depending on the specific defect and severity of
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symptoms.
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Atrial Septal Defect
vidence ree
Sinus Venosus Coronary Sinus

ASD Patent Foramen Primum ASD
= ASD post/sup to FO (o5en w
anom R pulm vn)
Ovale ASD = ASD via unroofed CS in LA,
o5en w an LSVC
= failure of FO to seal closed = ASD anterior to the FO

when LAP >RAP postnatally

Secundum
-‐Present in 15-‐25% of people.R4
See Anomalous Pulmonary
Venous Return Algorithm, -‐No Need to Intervene as always ASx. ASD See Atrioventricular Septal
Defect Algorithm, though
-‐Only indica\on to close is 2 strokes = ASD of the septum primum
though some evidence here Risks of PFOR4, R5 (but insufic data per AHA 2011 Guidlns) some evidence here may
may apply. -‐?if assoc w atrial septal aneurysm
-‐CLOSURE I RCT-‐ PFO closure in pt w apply.
(1-‐2% prevalence) stroke doesn’t reduce risk of 2nd stroke
-‐PFO & AS Anyrsm + Incr risk stroke (differs from many obsv studies)R13, R14
for pt <55y-‐ OR 3(CI95 2.3-‐4.2). If -‐Doesn’t reduce Migraines in MIST RCT
>55yo, OR is only 1.27 (CI95 0.8-‐2) for R16 though did w obs studiesR15. R17
PFO. Incr risk if PFO & AS An; -‐Might decr atr arrhyth risk per meta.R20
-‐No proven incr risk of strokeR17, R18
-‐If pt had a stroke, no incr risk for 2nd
stroke at 2yr f/u “Is there risk Small & ASx
Moderate
Large & Sx
-‐Eu study of pts <55yo, risk of 2nd
stroke was incr w PFO
to a PFO?
Other studies differ, No RCTs Should we

-‐See R17 for detail on xx evidence
close it?” c/s Cath Closure
Long Term NHx

-‐2nd ASD might spont close, but also get biggerR1 Mod-‐Large & +Sx
-‐Up to 20% close spont’ly, most by 4yoR1, w up to
ASx & <3mm
66% of small ASDs closing in other studiesR7 refs,
mainly d/o age at Dx
Symptoma\c ASD is an
Only follow up if indica\on to close, though Rx
-‐in pt Dx at <3mo if <3mm, nearly all close by symptoms occur or Long Term Px & NHxR1
can stall & treat Sx
“What is the 18mo, if 3-‐5mm 87% close by 12mo, if 5-‐8mm,
80% by 15moR10 exam changesR1, R10 -‐Diure\cs (Lasix)
-‐R heart volume ODà dilated RA & RV
-‐mural thrombi uncommon
chance this -‐One studyR7 of 104 pt w 2nd ASD >3mm at Dx, avg
4 yrs old at Dx w 3yr f/u:
-‐Sev RV dila\on can à RV forming apex, IVS
straightens so both ventricles are D shaped
will close
without
-‐of 34 small (3-‐6mm), at 3yr f/u 3 closed, 6 got
signif bigger (to 6 to >12mm); of 40 mod (6-‐12mm)
ASx & >3mm
“What will -‐TV and PV dila\on, w TR & PR, mildly thick
-‐Central Pulm Art Dila\on
at 3yr f/u 3 descr to <6mm & 8 incr to >12mm; of Follow Up at 12 & happen -‐LV size/mass remains Nl
surgery?” 30 large (>12mm), at 3yr f/u, all remained large
-‐overall, 65% incr in size, avg by 0.8mm/yr; 14%
15 months old or if
without
-‐Ch Vol OD to Lungsà medial hypertrophy of
arts/vnsà pulm htn, plexiform & thrombo\c
Sx startR1, R10
decr in size, no change in growth rate by Dx age,
ini\al size correlates w final size (r2 = 0.6)
surgery for a obst lesions; may worsen other dz pt may have
(COPD etc)
-‐Similar results in diff study 56% close if 4-‐5mmR11
-‐Up to 24% of newborns had an ASD of some sort,
large ASD?”
“Why go to
and 92% closed by 1yo-‐ more likely if <7-‐8mm
diam, and younger at DxR1 ASx & Qp:Qs <1.5 ASx & Qp:Qs >1.5
surgery if the Long Term Px
-‐benign unless very largeR1
Con\nue to monitor Indica\on to close at pre-‐
school age (by 4yo)
child doesn’t -‐may be ASx through >50s w a moderate ASDR1 serially
(Qp by exam, echo, cath) Older Adult with ASDR3 & R12
have -‐CHF uncommon early on, but by >40yo.R1
-‐Atrial Aryth are about 10% by 40yo, 20% overallR2 -‐RCT 473 Mexicans 40-‐70yo w any ASD type, NYHA 1-‐2
symptoms?” -‐Afib/fluaer – 13% at 40, 50% at 60yoR1
-‐If untreated, 5-‐10% of pt (F>M) get pulm vasc dz
-‐all with Qp:Qs >1.7, PAP <70; 7yr f/u avg
à  Surgery beêr than Rx Tx
usually at age >20yoR1 EndPt: ΣCV death, worsen HF, syst/pulm emboli, recur pulm “What are the
ASx & Arrhythmia in AdultsR2
infec\on, SVT, incr phtn >20% baseline risks to adults
with ASD?”
HR 1.99 (CI95 1.23-‐3.22), p MVA 1.85 (1.08-‐3.17) w R/F for
Adult with Asx ASD (any type, not PFO): Closure will worse outcome = incr age at Dx, PAP >35mmHg, or RxTx
reduce risk of arrhythmia in medium term (<5yr), but -‐2y endpt death: signif diff in MVA analysis only w HR 4, if
not long term (>5yr) per Meta data: adjust for age, PAP>35, prev afib/fluaer, CI <3.5
-‐Σ OR0.66 overall, 0.47 in med term (CI95 0.36-‐0.62), -‐One StudyR12, retro non random Surg vs Rx in pt Dx at >40yr
but long term OR 0.7 (CI95 0.65-‐1.07), but limited high Surgical Indica\ons: w Qp:Qs >1.5 à surg improves NYHA (RR0.2) and survival
quality long term data -‐wait \ll 4yo for elec\ve repair (pre-‐school, (95% vs 84% 10yr f/u, but no change in arrhyth or CVA, but
-‐Of high quality studies OR 0.7 (CI95 0.51-‐0.97) big enough to make it easier) Rx pts were older, and 20% Rx pt went to Surg, and 27% had
-‐for afib/fluaer OR 0.77 (CI95 0.63-‐0.95) -‐if an adult, might have earlier mortality (but systemic/suprasyst RVP…
àc/s closure, knowing no proven long term effect uncertain bc healthy ppl w ASD might be lost -‐Another studyR13, of NYHA I&II pt >25yo Qp:Qs>1.5, did not
to f/u in the study) show that surg improves Sx-‐ no diff in survival, stroke, HF

Cath Requirements:
-‐Pa\ent size & age
“Can we close Asx ASD w Qp:Qs >1.5:1 by cath/echo
Asx but murmur/fixed split S2 or orther
-‐Atrial Septal Rims: R25 (details xx by rim defic) it via cath?” signs/sx of Qp:Qs >1.5 Pre-‐Op Ques\ons
-‐Echo Eval:Lai Text
1. AV vlv rim |4. (RUPV rim) |7. CS rim Sx of FTT, CHF uncontrolled with meds
[ ] Defect loca\on & size
2. Ao rim |5. Post rim [ ] Rela\on to neighboring structures-‐ MV, RV, Syst/P Vns
3. SVC rim |6. IVC rim
“Does my [ ] Measure defect margins for ?transcatheter closure
[ ] Hemodynamics
[ ] flow direc\on, transseptal pressure gradient-‐ check mean gradient
child need [ ] check for HD load-‐ RA, RV enlargement, diastolic septal flaaening fr RV OD
surgery?”
Cath Closure Surgical Closure [ ] RV syst P by TR jet and systolic ventric septum configura\on
[ ] biventric fx
[ ] assoc lesions
-‐Amplatzer-‐ ideally 12-‐14mm rims, ideally Median Sternotomy Thoracotomy

for defects w 18-‐38mm diamR25 -‐preferred by Mavroudis groupR21 -‐7.4% get ipsi breast maldvp, w periareolar
-‐Ao rim must be at least 5mmR26 numpness/hypoesthesia in 39%, low but
-‐CardioSEAL Minimally Invasive SurgeryR19 present risk of atalectasis, R phrenic palsy,
-‐Helex septal occluder -‐5-‐7mm scar only, but limited exposure rib fxR9

-‐about 98-‐99% successful outcome (adult/ -‐One study of 23 pts show good outcome -‐incr risk of R phrenic n injury & air
peds mixed data)R1, R24, with subsequent embolusR21
improved RV dila\on etcR24

Cath Risks:
-‐Erosion causing tamponade, Ao fistula,
“What are the Surgical Risk:
-‐0.04% mortality for 2nd & sinus venosus ASD “If you close it,
aaributed to over or under sized which can risks to Cath & -‐see Cath Risk for comparison will my child be
incr contact w Ao root. Incidence unknown
but 0.2-‐0.3% reported.R22
Surgery?” cured?”
-‐Safer than Surg-‐ One study wkids & adults
4x less risk of major xx-‐ HF, AVB needing Long Term Px
97% survival at 5yrs postopR1
temp pacer, arryth needing cardioversion,
-‐90% survival at 10yrs postopR1
bleeding needing reop, PTx, etcR23
-‐74% at 30yrs post upR1
-‐14/751 Cath pts needed opera\on for (Ctrl pts had 99%, 98%, 85% survival) R1
malposi\on/embolizn -‐57% of pts >24yo had late CVAs compared to 15% if <24yo at surgical correc\onR1
-‐trivial/small resid shunt more common w -‐One study of pts w surgery for 2nd or Sn Vn ASD at Mayo in 1950-‐60s, no diff in 27yr
cath (8% vs 3%) but mainly w Cardioseal and survival if <25yo at surg, but if >25-‐41 then only 84% vs 91% in ctrl, and if >41 then
not Amplazter 40% survival vs 59% in ctrl w more late CHF, stroke, a-‐fib. 75% of all the pts were +Sx
-‐One study (adult/peds) showed 2/176 pts at surgery. R6
needed surg for resid ASD or device
malposi\on, 7% w arrhyth (SVT/VT), no
mortality
-‐One study: 1% postop xx needing surgR25
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Atrial septal defects & Partial anomalous venous connection
Definition
An atrial septal defect (ASD) is an opening in the interatrial -First closed in 1952, by John
septum, causing a left-to-right shunt and volume overload of Lewis (Minnesota) w
the right atrium and right ventricle. ASDs account for about 6 to hypothermia & inflow
10% of cases of congenital heart disease. occlusion
Most cases are isolated and sporadic, but some are part of a -First to use CP bypass
genetic syndrome (eg, mutations of chromosome 5, Holt-Oram (5/6/53), by John Gibbon
syndrome). -First to use purcutaneous
cath closure
-Now most closed by cath
Anatomy & Pathology

• Septum Secundum - thick, superior part, made fr infolded Associated lesions
atrial roof • PS - 10%
• Septum Primum - lower part, made of a thin flap of tissue, w • PAPVC - 7%
the lower edge fusing to the endocardial cushion. Goes • VSD - 5%
upwards and to the left of the limbus of the septum • ASD + rheumatic MS =
secundum (to bec ome the flap valve that closes the foramen Lutembacher syndrome
ovale) • MVP, MR 2ry cleft mitral
• At IVC os, there is the CS os & the eustachian valve valve.
• Crista Terminalis = thick part of the infolding of the lateral RA
wall running fr the sup to inf aspect.
Secundum ASD ASD Types:

=@ fossa ovalis (part of the septum primum) • Ostium Secundum (80%)
Sinus Venosus ASD • Ostium Primum (10%)
=@ jct of SVC and RA, more posterior & cranial to the superior • Sinus Venosus (10%)
limbic band • also - common atrium,
-near always assoc w PAPVR of the R sup PVn to RA. Tends to coronary sinus ASD, and
enter LA at jct of SVC and RA along the R'ward margin of the • PFO often have LSVC to
ASD (see pick). Sometimes it goes directly into SVC (near CS or sometimes direct to
azygous entrance) LA
-Rarely, the SVn defect is near the IVC orifice, and it is assoc w
a R inf PVn anomalous entry
Ostium Primum Defect
-Crescent shaped defect at inf part of the septum, just adjacent
to the AV vlvs
-it is part of a CAVC defect, so aka partial AVSD/AVCD. assoc w
cleft ant leaflet of the left AV vlv
Common Atrium
-failure of entire atrial septum to dvp; assoc w heterotaxy
syndrome
Patent Foramen Ovale
-no defic in septum primum, and limbus is normal, but the flap
never closed
-in true PFO (as oppose to ASD), it only opens when P o nR is >
P on L, e.g. w cough/valsalva
Unroofed Coronary Sinus
-allows for LA to RA shunt, though septum itself is in tact
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Pathophys
• L è RS usually bc RV more compliant than LV è Qp:Qs >1
• Large ASD can èQp:Qs >4:1
• rarely assoc w cyanosis fr LèR bc the pt had a big eustachian
vlv that directs Q thru ASD to L side (like prenatally)...
• rarely have cyanosis fr an unroofed CS
• if pt has common atrium, then --> mixing enough to cause
cyanosis
NHx
• May get a-flutter & a-fib long term
• May get rv dysfx, pulm htn, paradoxical emboli, and then
eventual chf
• Mean age at death w/o surgery is 36 years in pts w signif asd
• Unlikely to close on own if >8mm
• Likely to close on own if <4mm
• Unlikely to close after 4yo
• Unlikely to get bacterial endocarditis, so no need for SBE ppx
• Usually ASx, just hear a murmur fr relative PS, & hear fixed
split S2 bc R side needs mroe time for Qp...
• +diastolic rumble in some fr relative TS
• RV vol OD and pulm vasc bed OD is well tolerated for years,
so it is rare for infants to have CHF fr an ASD alone, and less
likely to get pulm vasc dz than VSD or AVSD pts
• 10-25% of adults w untreated ASD had phtn (phtn = PAP
>30mmHg)
• 5-15% had high PVR
• but it was mainly with Sinus Venosus defects than 2nd ASD
(16% vs 4% of total group)
• If pt did get phtn to the pt of R-->L shunting, then closure is
c/i xx
• Late mortality in untreated ASD pts is CHF & arrhythmias
• Mayo clinic study found 55% chance of late a-fib if closed
>44yo vs 4% if closed by 11yo (ref12)
• èè Rec closing ASDs
Treatment
Indication to close:
• ASx ASD w cath/echo evidence of Qp:Qs 1.5:1 or more
• usually, if pt has murmur/fixed split S2 or other Si/Sx then it
is >1.5:1 shunt
• pref to close before kindergarden for convenience
• long term Px worsens if you delay it till Sx or adulthood.
Cath Closure for 2nd ASD only

• Amplatzer
• CardioSEAL
• Helex septal occluder
• but must have the right rims for it, #1 reason for surgical ref
is poor inf rim)
• xx of cath closure- device malposition & dislocation
Surgical Closure Alt 2nd ASD Techniques:
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95
1)-Median Sternotomy - many can do it w/o opening the entire directly suture the edges of the
sternum defect, or use PTFE patch, or
-Right Thoracotomy - alt approach, but incr risk of air use Dacron (polyester)
embolus and R phrenic n injury (not used by Mavroudis group) Direct suture w 2 layers of
running suture..., though use of
2) Divide thymus at midline; no need to resect it
a patch can reduce recurrence
3) Autologous Pericardial Patch harvest
-At start of case, harvest it, and use stay sutures to retract
the corners and place in a saline solut'n
4) CP Bypass- Ao cannula and 2 venous cannulas
-for 2nd ASD, cannulate the RAA first, and then later advance
it to SVC
-Cannulate IVC at jct w RA, low enough so that if there's no
inf rim of the ASD, you can get good exposure in that area
-if Sinus Vn Defect, must cannulate SVC directly w a RA cath
at jct of innom vn
5) Cool pt to 32C
6) For an AVSD, vent at the R sup PVn..., otherwise they don't
vent...
7) Caval Tapes at SVC & IVC placed
8) Cardioplegia given & caval tapes are snared
9) 2nd ASD: RA incision fr RAA to IVC. Don't cross the crista
terminalis (to maintain SA nd to AV nd conduction fibers)
SnVn ASD: RA incision fr RAA tip twd SVC-RA jct, and if
needed extend across the jct of SVC to RA, along the R lateral
margin of the SVC
10) 2nd ASD: use sucker for Q through ASD fr LA. If much Q,
c/s a PDA or high pump flow
• Don't suck the LA empty bc a full LA ensures no air entering
it fr RA...
• Suture the patch starting at inf aspect of ASD, near IVC, and
ensure you don't bite into estuach. Vlv (if you suture
eustachian vlv to septum secundum, you might get an
obligatory IVC to LA shunt!)
• Then suture superiorly first near crista terminalis, then near
CS. By the CS, don't take deep bites or you might injure the
AV nd. Complete the sutures at highest pt of the L and RA
jct
• Before knotting the suture, make an opening at the jct of
patch and ASD and make pt Valsalva to push blood and air fr
LA/pvns thru ASD; note some bubbling as L side is de-
aired. As pt is valsalva'd (once the bubbles resolve), then
pull the suture tight and tie the knot. Then repeat the
valsalva to assess for leaks at teh patch
• Then decompress the L side of the heart by venting thru the
cardioplesia needle site
• Don't vent too strongly or you will pull air into the L side
• Close the RA incision, and de-air before releasing the cross
clamp
• Rewarm pt as you close the RA incision
• Wean from bypass, remove venous cannulas, and use
modified ulatrafiltration for 10 min to decr need for prbc,
then reverse the heparin w protamine, and remove the Ao
cannula
• Extubate in OR or next day
9
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Alt Sn Vn ASD approach:

Sinus Venousus ASD: Flaps of RAA or direct
anastomosis of SVC to RAA for
• Nearly always need a patch bn SVC and RA, and direct suture complex defects to avoid SA nd
can è SVC or R Sup pvn stenosis injury (Ref 30)- transect SVC
• TEE should be used for all Sn Vn ASD, check at end for and oversew above R PVn
residual defect insertion, then anastomose the
• Beware proximity of SA nd SVC to the RAA and close the
ASD w a patch..., others use
• Beware the R phrenic n, on the other side of the pericardium
RAA to make an atriocavoplasty
next to the R lateral part of the SVC
• 20-40% can be closed w a simple pericardial patch
• Start suture line bn the R Sup pvn orifice and the SVC
orifice, then continue it around the rest of the ASD. May
need to enlarge the ASD so that the RUPV isn't stenosed- do
this w incision twd FO and rsxn of part of the limbus.
• Ensure sutures are superficial at post part of SVC, to avoid
SA nd injury
• If the PVn enters the SVC, then the patch might need to
extend into teh SVC itself to partition it into R and L
side. Use a two patch technique to prevent billowing of the
patch, which would cause SVC stenosis. In doing this, when
you incise at the SVC-RA jct, make it as lateral as possible to
avoid SA nd injury.
• Don't confuse the azygos vn w the anom R sup PVn (azygous
usually has dark bld!, and PVn nearly bloodless bc pt is on
CPB)
•
Ostium Primum ASD:

Technical Pitfalls
• Vent thru R Sup pvn • may be hard to see inf
• Cardioplegia then open RA part of ASD, espec if IVC
• Then remove vent fr MV orifice and LV, and place in post LA cannula is too high
• Irrigate L AV vlv w cold saline to assess for MR • if you mistake a large
• Close cleft in L AV vlv w interrupted sutures, then replace the eustachian valve for the
vent into the LV lower edge of teh ASD,
• Some don't close the cleft if no regurg then you might cause an
• Start suturing pericardial patch at pt where the leaflets bridge IVC to LA shunt
the IVS crest, then suture clockwise twd CS (taking • if LSVC to CS, c/s a L
superficial bites there to avoid AV nd). Then bring a 2nd Glenn when you close the
suture line counterclockwise CS roof defect...
Prognosis & Surgical Outcome Cath Closure Outcome

• No death & no recurrence at their institution w 1/2 suture About 98% success
and 1/2 pericardial patch closure
• No change in long term survival fr nl ppl.... Surgical Outcome in Older
• in their results w sinus venosus defect- r pvn stenosis in Pts
<10%, 7% w sa nd dysfx Higher risk, so controversial
• Ostium primum asd results- some get mr, av block w pacer, whether to Tx, but on the
LVOTO; ones tudy 98% survival at 10 yrs, but 9% needed whole it seems to improve
reoperation for subao obstruction, l av vl regurg survival and NYHA class...
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Partial anomalous pulmonary venous connection (PAPVC)

Definition
PAPVC is drainage of some or all the pulmonary veins in right
atrium or its tributaries. The hemodynamic changes and clinical
manifestations of this anomaly and atrial septal defects are
almost identical
General Pathologic Considerations • In ASD/PAPVC, shunting is left

to right initially
Morphology of PAPVC
• Some small ASDs, often just a
a. The most common type of partial anomalous drainage is stretched patent foramen
associated with a sinus venosus defect. ovale, close spontaneously
b. The right upper and sometimes the middle pulmonary vein during the first few years of
may be connected to the SVC at the junction of the SVC and life.
the right atrial junction. • Persistent moderate-to-large
c. In rare instances, a right superior pulmonary vein may be ASDs/PAPVC result in large
connected to the SVC in the absence of a sinus venosus shunts, leading to right atrial
defect. and right ventricular volume
overload. If unrepaired, these
d. All pulmonary veins from the right lung may drain to the
large shunts may lead to
right atrium; the orifices of individual veins may be close pulmonary artery
together or separated. Alternatively, the superior pulmonary hypertension, elevated
vein may drain to the SVC and the others to the right pulmonary vascular
atrium. resistance, and right
e. Scimitar syndrome: this is a rare anomaly in which all right ventricular hypertrophy.
pulmonary veins may also drain to the IVC. The anomalous • Atrial arrhythmias, such as
pulmonary venous trunk is usually anterior to the right hilum supraventricular tachycardia
and enters the IVC just above or below the diaphragm. The (SVT), atrial flutter, or atrial
fibrillation may also occur
orifice of the pulmonary veins is usually very close to the
later.
orifices of the hepatic veins. Scimitar syndrome is often • Ultimately, the increase in the
associated with right lung hypoplasia and dextroposition of pulmonary artery pressure
the heart. The blood supply to the part of the right lung may and vascular resistance may
be via aortopulmonary collateral vessels originating from the result in a bidirectional atrial
descending aorta. On occasion, true sequestration may be shunt with cyanosis during
associated with the scimitar syndrome. adulthood (Eisenmenger
f. Anomalous drainage from the left lung may be either to the reaction).
innominate vein or via an anomalous vertical vein or to the
coronary sinus
• Most patients are asymptomatic.
• Larger shunts may cause exercise intolerance, dyspnea
during exertion, fatigue, and atrial arrhythmias with
palpitations.
• Paradoxical embolization, often associated with arrhythmias,
may lead to cerebral or systemic thromboembolic events,
such as stroke.
• Rarely, when an ASD is undiagnosed or untreated,
Eisenmenger syndrome develops.
• Auscultation typically reveals a grade 2 to 3/6 midsystolic
(ejection systolic) murmur and a widely split, fixed S2 at the
upper left sternal border in children.
• A low-pitched diastolic murmur, in large shunts (due to
increased tricuspid flow), at the lower sternal borders.
• A prominent right ventricular cardiac impulse, manifested as
a parasternal heave or lift, may be present.
11
97
98
Diagnosis
• Chest x-ray shows cardiomegaly with dilation of the right
atrium and right ventricle, a prominent main pulmonary
artery segment, and increased pulmonary vascular markings.
• ECG may show right axis deviation, right ventricular
hypertrophy, or right bundle branch block
• Echocardiography confirms the diagnosis
Management
• Most small centrally located ASDs (< 3 mm) close
spontaneously and many defects between 3 mm and 8 mm
close spontaneously by age 3 years.
• Ostium primum ASDs and sinus venosus ASDs do not close
spontaneously.
• Observation: in cases of asymptomatic children with a small
shunt
• Moderate to large ASDs with evidence of right ventricular
volume overload on echocardiography should be closed,
typically between ages 2- 6 years
• Transcatheter closure with various devices (eg,
Amplatzer® or Gore HELEX® septal occluder) is preferred
when appropriate anatomic characteristics, such as adequate
rims of septal tissue and distance from vital structures (eg,
aortic root, pulmonary veins, tricuspid annulus), are present.
• Surgical repair: in sinus venosus and ostium primum defects
and PAPVC.
required only for the first 6 mo after repair or if there is a
residual defect adjacent to a surgical patch.
12
98
99
FIGURE 4−4.
13
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100
Ventricular Septal Defect (VSD)

Definition
A ventricular septal defect (VSD) is an opening in the
interventricular septum, causing a shunt between ventricles.
VSD is the 2nd most common congenital heart anomaly after
bicuspid aortic valve, accounting for 20% of all defects.
Pathophysiology Kapoor L, Gan MD, Das MB,
• Morphology: VSDs are classified by location: Mukhopadhyay S, Bandhopadhyay
a. Perimembranous (70 to 80%): they are defects in the A. Technique to repair multiple
membranous septum adjacent to the tricuspid valve and muscular ventricular septal defects.
they extend into a variable amount of surrounding J Thorac Cardiovasc Surg. 1999
Feb;117(2):402-4.
muscular tissue
Seddio F, Reddy VM,
b. Trabecular muscular (5 to 20%): they are completely
McElhinney DB, Tworetzky W,
surrounded by muscular tissue and may occur anywhere Silverman NH, Hanley FL. Multiple
in the septum. ventricular septal defects: how and
c. Subpulmonary outlet (supracristal or doubly committed when should they be repaired? J
subarterial) (5 to 7%): they occur in the ventricular Thorac Cardiovasc Surg. 1999
septum immediately under the pulmonary valve. They Jan;117(1):134-9; discussion 39-40.
are frequently associated with aortic leaflet prolapse into Kitagawa T, Durham LA 3rd,
the defect, causing aortic regurgitation. Mosca RS, Bove EL. Techniques
d. Inlet (5 to 8%): They are bordered superiorly by the and results in the management of
tricuspid annulus and are located posterior to the multiple ventricular septal defects. J
membranous septum. These defects are sometimes Thorac Cardiovasc Surg. 1998
referred to as atrioventricular septal-type defects. Apr;115(4):848-56.
• Malalignment type refers to its relation to the alignment Brauner R, Birk E, Sahar G,
of trabecular to conal septum and aorta seems displaced Blieden L, Vidne BA. Surgical
relative to VSD. Anterior malalignment is associated with management of ventricular septal
TOF, posterior malalignment is associated with defect with aortic valve prolapse:
clinical considerations and results.
interrupted aortic arch or coarctation, and rotational
Eur J Cardiothorac Surg.
alignment is associated with Taussig-Bing anomaly
1995;9(6):315-9.
• The magnitude of the shunt depends on defect size and
downstream resistance (ie, pulmonary outflow tract
obstruction and pulmonary vascular resistance).
• Blood flows easily across larger defects, which are thus
called nonrestrictive; pressure equalizes between the
right and left ventricles and there is a large left-to-right
shunt.
• Assuming there is no pulmonic stenosis, over time, a
large shunt causes pulmonary artery hypertension,
elevated pulmonary artery vascular resistance, right
ventricular pressure overload, and right ventricular
hypertrophy. Ultimately, the increased pulmonary
vascular resistance causes shunt direction to reverse
leading to Eisenmenger syndrome.
• Smaller defects, also referred to as restrictive VSDs, limit
the flow of blood and the transmission of high pressure to
the right heart. These VSDs result in a relatively small
left-to-right shunt, and pulmonary artery pressure is
normal or minimally elevated. Heart failure (HF),
pulmonary hypertension, and Eisenmenger syndrome do
not develop.
14
100
101
• Symptoms depend on defect size and magnitude of the
left-to-right shunt.
• Children with a small VSD are typically asymptomatic and
grow and develop normally.
• In children with a larger defect, symptoms of HF (eg,
respiratory distress, poor weight gain, fatigue after
feeding) appear at age 4 to 6 wk when pulmonary
vascular resistance falls.
• Frequent lower respiratory tract infections may occur.
• Eventually, untreated patients may develop symptoms of
Eisenmenger syndrome.
• Auscultatory findings vary with the size of the defect.
• Small VSDs typically produce murmurs ranging from a
grade 1 to 2/6 high-pitched, short systolic murmur to a
grade 3 to 4/6 holosystolic murmur (with or without thrill)
at the lower left sternal border. The precordium is not
hyperactive, and the 2nd heart sound (S2) is normally
split and has normal intensity.
• Moderate to large VSDs produce a holosystolic murmur
that is present by age 2 to 3 wk; S2 is usually narrowly
split with an accentuated pulmonary component. An
apical diastolic rumble (due to increased flow through the
mitral valve) may be present
• In moderate high-flow VSDs, the murmur is often very
loud and accompanied by a thrill (grade 4 or 5 murmur)
• With large defects allowing equalization of left ventricular
and right ventricular pressures, the systolic murmur is
often attenuated.
• Findings of HF (eg tachypnea, gallop, crackles,
hepatomegaly) may be present.
Diagnosis
• Chest x-ray:shows cardiomegaly and increased pulmonary
vascular markings, if the VSD is large
• ECG shows right ventricular hypertrophy or combined
ventricular hypertrophy and, occasionally, left atrial
enlargement, if the VSD is large.
• Echocardiography establishes the diagnosis and can provide
important anatomic and hemodynamic information, including
the defect's location and size and right ventricular pressure
Management Meijboom F, Szatmari A, Utens
E, Deckers JW, Roelandt JR, Bos E,
a) Small VSDs, particularly muscular septal defects, often close Hess J. Long-term follow-up after
spontaneously during the first few years of life surgical closure of ventricular septal
b) Medical therapy: defect in infancy and childhood. J
• Diuretics, digoxin, ACE inhibitors Am Coll Cardiol 1994 24(5):1358-
• To control symptoms of HF before cardiac surgery or to 64.
temporize infants with moderate sized VSDs that seem Backer CL, Winters RC, Zales
likely to close spontaneously over time VR, Takami H, Muster AJ, Benson
c) Surgical repair DW Jr, Mavroudis C. Restrictive
• If infants do not respond to medical treatment or have ventricular septal defect: how small
poor growth, surgical repair is often recommended during is too small to close? Ann Thorac
the first few months of life. Surg 1993 56(5):1014-8.
• Even in asymptomatic children large VSDs should be Kidd L, Driscoll DJ, Gersony
repaired, usually within the first year of life, to prevent WM, Hayes CJ, Keane JF, O'Fallon
15
101
102
later complications WM, Pieroni DR, Wolfe RR,

d) Device closure of VSD may be considered Weidman WH. Second natural
• Especially if the VSD is remote from the tricuspid valve history study of congenital heart
and the aorta defects. Results of treatment of
• Indications for catheter device closure of VSD include patients with ventricular septal
muscular VSD, residual defects after prior attempts at defects. Circulation 1993 87(2
surgical closure, restrictive VSDs with a significant left-to- Suppl):I38-51.
right shunt, Okubo M, Benson LN,
Nykanen D, Azakie A, Van Arsdell
e) Endocarditis prophylaxis is not needed preoperatively and is
G, Coles J, Williams WG.
required only for the first 6 mo after repair or if there is a Outcomes of intraoperative device
residual defect adjacent to a surgical patch. closure of muscular ventricular
septal defects. Ann Thorac Surg.
2001 Aug;72(2):416-23.
Pretre R, Benedikt P, Turina
MI. Modified approach to close
multiple apical ventricular septal
defects. Eur J Cardiothorac Surg.
1999 Dec;16(6):683-5.
16
102
103
FIGURE 4−21. Algorithm for the management of VSD according to the physiological
categories.
17
103
Ventricular Septal Defect
vidence ree
VSD & Pregnancy (study of 81 pts, 149 pregnancies)
-‐low rate of CV event w pregnancy
-‐Repaired Mod-‐Large VSD:
w/o pulm htn
-‐higher risk of premie labor (AOR 4.02) relaPve to
unrepaired & higher risk of SGA (AOR 4.09) relaPve
to repaired, but same risk compared to Nl pts, unsure
why; ?co-‐morbidiPes in pts who needed closure of
Restric1ve vs. Non-‐Restric1ve? VSD…
-‐No precise criteria agreed on. Membranous Muscular Juxta-‐Arterial -‐Unrepaired Mod-‐Large VSD
-‐higher pre-‐eclampsia (AOR 4.6 – 8.7% vs 1.8%)
-‐Cross-‐secPonal area of VSD vs. Ao
-‐VSD diameter vs. BSA VSD VSD VSD
relaPve to gen populaPon
-‐no CHF, stroke, TIA in either group, 1 unrepaired pt
-‐Peak Velocity of Q at VSD = adjacent to AV valve à = completely surrounded = adjacent to Arterial valves had IE post delivery
TV-‐Ao Valve ConPnuity by muscle (aka supracristal, conal)
Direc1on of Q?
-‐In Non-‐RestricPve VSD, Q d/o PVR:SVR
-‐LàR incr as PVR drops, and may be delayed
NHx of Large VSD without Tx -‐ 3-‐7% w PM VSD get DCRV (? If size
If PVR remains high.
CHF Sx postnatally as PVR makers); assoc w LVOTO up to 88%
-‐Eisenmenger’s Syndrome = If uncorrected and
drops, then incr PVR from -‐some get DCRV even w spont
paPent gets pulm. vasc dz, and PVR>SVR,
“What’s the risk of
Small & Large & Symptoma1c pulm vasc dz a@er 1-‐2yr, w RV VSD closure
Then see RàL Q at VSD.
Asymptoma1c Moderate failure; Eisenmenger a@er -‐8.6% get Ao vlv Prolapse in all VSD
not closing a mod-‐ 20yo, death by 40yo -‐5.5-‐9.4% get AR of all VSD types
large VSD?”
-‐Many get LH DilaPon w higher Qp
29/33 ASx paPents with a
-‐Pulm Vasc Dz a@er 1-‐2 yrs…
Small VSD and + Volume
OD on LV had z score fall -‐3.1% PM VSD have sub-‐Ao ridge
to <2yo by 8yr mean f/u. + LV Volume Overload
but no Pulm Hypertension
Long Term Px Likely Best to observe without Infant with Pulmonary
-‐Adults w small VSDs, Nl PA P, Nl Hypertension Eisenmenger’s Syndrome
surgical or cath closure.
ventric fx, no assoc xx à Nl Infant w Pulm Htn
exercise tolerance. -‐usually +FTT & CHF Sx -‐Avoid high alPtude bc of incr risk
-‐no restricPons needed (92) -‐ACE & DiurePc of hyperviscosity & arterial
-‐small VSD in PG women doesn’t
“What are -‐Surgery by 3mo
-‐Risks: renal failure & hypoxemia.
hypotension w ACEI especially (58)
incr CV risk during PG; -‐No evidence for venesecPon or

-‐Most Musc V SD close (~80%): One study: If
risks of Rx anPcoagulaPon.
-‐Must avoid pregnancy bc of fetal/
spont close, 93% close by 3yo
-‐Many PM VSD close: One study: If spont close,
Small Asx VSD w/o LV
Dila1on
Treatment?” “How effec1ve is maternal risk.
-‐Bosentan improves 6 min walk
96% clz by 6yr.
medical therapy?”
Likely best to observe without Moderate VSD +Sx test in RCT (BREATHE-‐5 study)

surgical or cath closure. -‐Sildenafil increased QoL & Sx
-‐ If no CHF, total 72% pts had spont closure,
23% of subpulm, 74% of PM, 85% of muscular Risk of HF by 1-‐6mo, but
-‐DiurePcs
“What is the
VSDs, w a mean age at f/u of 6.9yrs most respond to Rx, and
-‐Lasix Risk= hi Ca, renal xx Long Term Px
In one Japanese study, of 225 pts, any VSD size, most don’t need surgery.
-‐Spironolactone for K -‐Eisenmengers synd ptsà lowest pk O2
all Dx at <3mo:
-‐Surgical closure in 26% of pts-‐ 8% subpulm VSD, chance it will close sparing if needed

Risk of infundibular PS
consumpPon during exercise, and was a
on its own?”
18% PM VSD. -‐ACEI (Enalapril/Captopril) that may progress w Pme.
marker of Px (93)
-‐On avg, spont closed by 19mo. 96% of the PM for systemic AL reducPon -‐If + Eisenmengers then very high risk of
+ Aor1c Valve Prolapse maternal and fetal death and premie delivery
VSD’s closed occurred by 6yo. 93% of the Musc -‐Digoxin if DiurePc + ACEI 15-‐20% of pts w mod VSD
VSD closed by 3yo. dvp a large shunt and with pregnancy (94)

AorPc RegurgitaPon is Controversial decision pt; no RCTs. fail, but of ? efficacy
associated with worse must go to surgery for
-‐Of 183 pts w muscular VSD, none closed a@er 7 persisPng signif LàR
outcome post VSD repair
yrs (most close by 5yr) Recommend surgery if pt has a PM shunt a@er 1yo (M&A ref
with increased
-‐Of 107 pts w PM VSD, none closed a@er 5.2yrs reoperaPon (65)
VSD with more than trivial AR. 30-‐31)

Risk of Not Closing: At 3yr f/u in 450 pts w PM In study of pts w trivial to
-‐No mortality at 30yr f/u of 229 pts deemed not VSD, 6% had subAo ridge, mod VSD, 95% were
to need closure. 12% had Ao-‐vlv prolapse, NYHA Class I a[er 15yrs
-‐95% of these were ASx, 89% Nl LV size, 10% and 7% had AR.
borderline LV size
-‐4/229 pts (2%) had endocardi1s (0.03%/yr risk
for each pt) “What’s the risk of
not closing a small SBE PPx
-‐Not rouPnely indicated
Small PM VSD at teen (study of 220
16yo w ~6yr f/u)
VSD?” -‐Only if residual shunt a@er
transcath/surgical repair
-‐all restricPve, Qp:Qs<1.5, Pre-‐operaPve ConsideraPons -‐Stress importance of dental
7% needed closure
Ques1ons by Echo: hygiene in all pts
4% close spont’ly
4% had endocardiPs -‐size & locaPon Surgical Indica1ons
1% (2pt) died-‐ 1 of CHF p closure w VF; -‐relaPon to TV, Ao Vlv, Pulm Vlv -‐Large VSD + CHF Sx despite Rxà close by 3mo
& sudden death -‐PM VSD-‐ Ao and TV leaflets related; conducPon syst -‐Large VSD w CHF responding to Rxà close if
is post-‐inf to VSD unlike muscular VSDs… present a@er 6mo, espec if PVR >4Wu or Qp:Qs >2
-‐Assoc RVOTO or LVOTO? -‐Large VSD in older pt w pulm vasc dz-‐ do NOT close
-‐Assoc Ao Vlv prolapse? if PVR is 8Wu and no response to vasodilators.
When to Consider MRI? -‐RV Pressure -‐AorPc Prolapse or >trivial AR
-‐RV & LV loading -‐Conal VSD, regardless of size, bc high chance of Ao
-‐if poor echo images -‐LVESD and LVEDD prolapse by 5yo
-‐complex dz (e.g. DCRV) -‐Pressure grad at VSD -‐Bacterial EndocardiPs regardless of size
-‐Assess for CoAo, PA, PVn and IVC/SVC xx
-‐check Qp:Qs, PA
resistance Surgical Closure:

Cath Closure: Ventriculotomy Trans-‐ Pulmonary Trans-‐ Aor1c Valve Trans-‐ Atrial (TV) PA Banding Valved Patch
-‐RV-‐ uncommon; use if Valve -‐if need to correct -‐#1 for PM, Inlet, -‐rarely done now -‐allows only right to
-‐espec good for can’t get to RA fr PA -‐for conal defects assoc lesion-‐ Ao Muscular, LV-‐RA VSD -‐palliaPve le@ shunPng
muscular defects that -‐VSD extends sup’ly -‐must use patch to valvuloplasty w -‐beware of conducPon -‐mainly in infants -‐low operaPve
are hard to get to for into infundib septum close conal VSD to prolapse, assoc AS system and Ao vlv -‐for pts w many VSDs mortality (85)
surgeon -‐improve exposure if support prolapsing Ao damage or apical VSDs that -‐quesPonable benefit
obstrcPve infundib vlv can’t be repaired (86)
-‐PM VSD: ok for VSD bundle -‐some are dilatable or -‐for older pts w signif
<18mm diam, >2mm fr -‐can’t expose the inf disolvable, meant for phtn
Ao, or small VSD w h/o margin of conal defect pts w muscular VSDs,
IE well so won’t need 2nd
-‐Not good for inlet VSD LV Approach-‐ rare-‐ for surgery
(VSD w/o post muscle certain trabecular VSDs
rim) or subarterial VSD w mulPple apical
“How likely is it

bc of proximity to AV defects (easier to patch Complica1ons, Outcomes, and Long Term Px
and semilunar vlvs fr L surface bc it is -‐0% mortality in older pts, higher in infants previously
-‐likle data on infants, smoother than RV) -‐excepPon: Swiss Cheese VSD 5-‐10% mortality but improving (Japanese study) that the surgery
-‐ReoperaPon needed in 18%
succeed?”
and about 25% of ptw
PMVSD need it closed -‐Significant Residual VSD in 1%
as an infant -‐ConducPon System Injury

Cath
Risks & Outcome:
-‐transient arrhythmia from cardioplegia
-‐RBBB (uncommon w trans-‐atrial), common w ventriculotomy; ? No future xx from it “What are the
PM VSD:
-‐Series of 27 pts: successful placement in 25, no residual Q
-‐Complete Heart Block needing pacer in 1-‐2% pts – bc of sutures through AV Nd/His bundle
-‐TV injury
risks of surgery?”
in 23 -‐Ao Valve injury – suture through it, incision through it
-‐another study showed 12/15 pts successful closure -‐Incomplete VSD closure if poor exposure, or bc disrupted sutures thru immature/diseased “What is the
-‐AR in 1/6 pts, embolizaPon in 1/13 pts
-‐TS & TR in 1 pt each
myocardium (reduced w TEE)
-‐CPB ComplicaPons-‐ neuro xx related to circ arrest Pme
prognosis a[er
-‐95% success rate in study of 430 pts

-‐Normal QoL reported (83), but may have behavioral & school performance problems in early surgery?”
childhood (83)
-‐Complete HB in 1-‐2%-‐ transient, transient BBB in 2.8%
-‐if phtnà self restrict amt of exercise

104
Congenital Coronary Artery Anomalies & Coronary artery

fistula
Pathophysiology
A) Minor Coronary Anomalies
• Clinically and hemodynamically insignificant anomalies due
primarily to an abnormal origin from the aorta or unusual
distribution.
• Circumflex artery arising from the right coronary artery or
sinus. Most common anomaly (0.5%).
• Left anterior descending from right coronary artery or right
sinus. It may be associated with transposition.
• Right coronary artery from noncoronary sinus. It's most
common coronary artery anomaly associated with Tetralogy
of Fallot (2%)
• Multiple coronary ostia
B) Major Coronary Anomalies

• It's hemodynamically Significant Coronary Artery
Anomalies
1. Anomalous origin of a main coronary artery from the
aorta
• Left main coronary artery from right sinus
• Right coronary artery from left sinus (more common)
• Single coronary ostia
• Clinical events related to course between pulmonary artery
and aorta resulting in compression, stretching or angulation
(especially during exercise)
• Treatment is revascularization or unroofing of intramural
segment of the anomalous coronary artery
2. Coronary artery aneurysms
• Congenital (rare)
• Acquired: Atherosclerotic more common (3-5%),
polyarteritis, Kawasaki
• Complications: Ischemia, thrombosis, distal embolization,
infarction
3. Coronary artery atresia/hypoplasia
• Severe LV dysfunction and sudden death,
• Not surgically correctable
4. Coronary artery stenosis (occurs with other congenital
lesions)
5. Coronary artery fistulas (most common)
6. Anomalous origin of left or right coronary artery from the
pulmonary artery
7. Acquired coronary anomaly: post surgical manipulation of
coronary artery as after Ross procedure or arterial switch
operations.
8. Anomalous Coronary Sinus (CS)
• Persistent L SVC
• Anomalous PV to CS
• Coronary artery to CS fistula
• Absence of CS
• Atresia of CS ostia - ass. with L SVC
• CS diverticula - ass. with WPW
18
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105
• CS septal defect
• Patients may present with aborted sudden death, chest pain,
arrhythmia, LV dysfunction, or exercise-induced presyncope
or syncope.
• A continuous murmur may be present in cases of coronary
artery fistula
• Cardiomegaly and mitral regurgitation are present in ALCAPA
Diagnosis
• Echocardiography
• Non invasive ischemia provocation testing
• Cardiac Catheterization and Coronary intravascular
ultrasonography have the potential to delineate mechanisms
of potential flow obstruction and are increasingly part of
diagnostic and therapeutic algorithms
• Coronary CT or MRI for more definitive definition of coronary
course.
Congenital Arteriovenous Fistulas

Definition
It is a direct communication between a coronary artery and any
of the four cardiac chambers, coronary sinus, SVC, pulmonary
arteries or veins.
Morphology
A) Right coronary most commonly involved (55%)
B) Left anterior descending (35%), combined (5%)
C) Site of connections
• Right ventricle 40%
• Right atrium 30%
• Pulmonary artery 20%
D) Fistulous opening most commonly is single
Management
According to size, complexity of the fistula and the origins of the
coronary arteries surgical treatment With or without bypass or
interventional catheterization are considered.
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106
Anomalous Origin of Left Coronary Artery from the Pulmonary

Artery (ALCAPA)
Definition
• Left main/Left anterior descending/or circumflex coronary
artery arising from main pulmonary artery or right pulmonary
artery
• Right coronary artery is normal
• Site of origin in the pulmonary artery is left or posterior cusp
• 65% of infants die within one year from CHF
• Mitral valve regurgitation caused by papillary muscle
ischaemia/ infarction may be present. This situation is known
as Bland–White–Garland syndrome
Management
Surgical repair is to establish a two coronary system.
• Left coronary artery transfer
• Subclavian to left coronary anastomosis
• Tunnel operation (Takeuchi repair)
• Coronary bypass grafting
• Ligation of left coronary artery
Congenital anomalies of Sinus of Valsalva

Definition
A localized weakness of the wall of the sinus of Valsalva leads to
focal bulging of the coronary sinus that may rupture into an
adjacent cardiac chamber, creating an aortocardiac fistula. This
is distinct from the diffuse dilitation of all sinuses seen in
patients with connective tissue disorders (i.e., Marfan
syndrome).
Pathophysiology van Son JA, Danielson GK,
Schaff HV, Orszulak TA, Edwards
• Abnormalities of the sinus of Valsalva may be congenital or WD, Seward JB. Long-term
acquired. outcome of surgical repair of
• The basic lesion is thinning of the wall of the aortic sinus just ruptured sinus of Valsalva
above the annulus. This may be the result of deficiency of aneurysm. Circulation 1994 90(5 Pt
elastic and muscular tissue in the aortic root. 2):II20-9.
• The aneurysm develops in this weakened area; eventually it Thirty-one patients had surgical
may rupture into one of the lowered-pressure cardiac correction with no operative
chambers, usually the right ventricle, less commonly the mortality and 95% survival at 20
right atrium, pulmonary artery, and very rarely the left years. All patients who required
atrium and ventricle. Very rarely it may be intrapericardial reoperation had right sinus-RV
rupture. fistulas; the authors recommend the
• Congenital aneurysms of the sinus of Valsalva are confined to transaortic approach for inspection
one aortic sinus, usually the right, less commonly the and repair of the aortic root.
noncoronary sinus and rarely from the left sinus. Hamid IA, Jothi M, Rajan S,
• A fistula may also result from rupture of an acquired Monro JL, Cherian KM. Transaortic
repair of ruptured aneurysm of sinus
aneurysm of the aortic sinus, from penetrating trauma, or
of Valsalva. Fifteen-year experience.
from congenital tracts in otherwise normal sinuses.
Journal of Thoracic and
• Rupture of a congenital aneurysm nearly always produces a Cardiovascular Surgery 1994
fistula between the aorta and one cardiac chamber, whereas 107(6):1464-8.
rupture of an acquired aneurysm is usually extracardiac, into
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107
the pericardial, mediastinal or pleural space. This series of patients also had
• Rupture is most common in 3rd or 4th decade low mortality. Use of the transaortic
• Endocarditis is present in 10%, although unclear whether this approach allows closure of an
causes the aneurysm or develops because of it associated VSD, which is commonly
• Associated anomalies found in these patients.
1. VSD Dev V, Goswami KC,
· 30-50% of patients Shrivastava S, Bahl VK, Saxena A.
· Juxtaarterial (supracristal) - leftward right sinus; most Echocardiographic diagnosis of
aneurysm of the sinus of Valvsalva.
common type
American Heart Journal 1993
· Juxtaaortic (perimembranous) - right 1/3 of right sinus 126(4):930-6.
· Hinge line of aortic leaflet separates aneurysm from VSD Sources for further reading
2. Aortic valve abnormalities Textbook Chapters
· about 20% of patients Chapter 39: Sinus of Valsalva
Aneurysm. Pediatric Cardiac
· AI secondary to prolapse usually associated with VSD Surgery (Mavroudis and Backer),
· Bicuspid valve also occurs 2nd ed., 516-520.
Chapter 21: Congenital
3. Pulmonic stenosis Aneurysm of the Sinus of Valsalva.
Cardiac Surgery (Kirklin and
· Usually subvalvular related to aneurysm in RVOT Barratt-Boyes), 2nd ed., 825-840.
· May also be due to RVOTO developmental abnormality, such
as TOF
4. Other lesions
· Coarctation
· PDA
· ASD
• Male predominance (80%)
• Clinically silent lesion until rupture
• 35% on rupture will have acute symptoms: precordial pain
and breathlessness
• 45% have gradual onset of dyspnea
• 20% have no symptoms and are diagnosed by new murmur
• Some patients have a latent period of weeks or years until
signs of right heart failure develop
• Severe symptoms are infrequent at time of rupture related to
small size of fistula or VSD
• Symptoms are worse if there is associated AI
• Characteristic murmur (continuous) and wide pulse pressure
Diagnosis
• ECG = LVH, RBBB, CHB
• Echocardiogram;
• Angiography useful to identify associated lesions
Management
• Surgical repair.
• The principle of surgery is simple.
• The defect in the aortic wall is closed,
• The aneurysm is resected,
• The fistula tract is obliterated.
• All this is usually accomplished by a combined approach of
opening the chamber into which the aneurysm protruded or
ruptured.
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108
Patent Ductus Arteriousus (PDA)

Definition
It's a communication between upper descending aorta distal to the
origin of left subclavian artey and main PA. It results of patency of
fetal ductus arteriosus. It is derived from distal part of 6th aortic
arch.
Pathophysiology
• PDA accounts for 5 to 10% of congenital heart anomalies; the
male:female ratio is 1:3
• In most patients, it is on the left side. If a right aortic arch is
present, the ductus may be either on the right or on the left;
very rarely is it bilateral.
• The media of the ductus consists mainly of smooth muscle that
is arranged spirally, and the intima is much thicker than that of
the aorta.
• Ductal closure:
Ø Functional closure: At birth, the rise in Pa o 2and decline in
prostaglandin concentration result in contraction of the
medial muscle causing shortening of the ductus and its
closure, typically beginning within the first 10 to 15 h of life.
If this normal process does not occur, PDA results
Ø Permanent closure: Folding of the endothelium and
proliferation of the subintimal layers causes ductal closure,
usually during the first 2–3 weeks of life. Closure begins at
PA towards aorta
• Prostaglandins E1, E2 and prostacyclin delay the
constriction of the ductus arteriosus at hypoxaemia.
However, vasoactive substances as acetylcholine,
bradykinin, and norepinephrine have all been shown to
produce constriction of the isolated ductus arteriosus
• Physiologic consequences depend on ductal size.
Ø A small ductus rarely causes symptoms.
Ø A large ductus causes a large left-to-right shunt. Over time,
a large shunt results in left heart enlargement, pulmonary
artery hypertension, and elevated pulmonary vascular
resistance, ultimately leading to Eisenmenger syndrome
• PDA aneurysms are rare
a. Infantile:
• Pulmonary end closed with marked narrowing
of the aortic end
• Usually involve the central portion
• Spontaneous regression-usually resolve
without therapy
b. Childhood
• Aortic end patent and closed pulmonary end
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109
• Has potential for rupture and death

• Clinical presentation depends on PDA size and gestational age at
delivery.
• Infants and children with a small PDA are generally
asymptomatic;
• Infants with a large PDA present with signs of HF (eg, failure to
thrive, poor feeding, tachypnea, dyspnea with feeding,
tachycardia).
• Premature infants may present with respiratory distress, apnea,
or other serious complications (eg, necrotizing enterocolitis).
• A grade 1 to 3/6 continuous murmur is heard best in the upper
left sternal border; typically has a different pitch in systole and
diastole.
• Full-term infants with a significant PDA shunt have full or
bounding peripheral pulses with a wide pulse pressure. A grade
1 to 4/6 continuous murmur is characteristic. If the murmur is
loud, it has a “machinery-sounding” quality.
• An apical diastolic rumble (due to high flow across the mitral
valve) or gallop rhythm may be audible if there is a large left-to-
right shunt or HF develops.
• Premature infants with a significant shunt have bounding pulses
and a hyperdynamic precordium. Murmur occurs in the
pulmonary area; the murmur may be continuous, systolic with a
short diastolic component, or only systolic, depending on the
pulmonary artery pressure.
Diagnosis
• Chest x-ray and ECG
• If the shunt is significant, chest x-ray shows prominence of the
left atrium, left ventricle, and ascending aorta and increased
pulmonary vascular markings; ECG may show left ventricular
hypertrophy.
• Echocardiography
• Cardiac catheterization
• Usually reserved for patients suspected of having increased PVR
(If PVR equals SVR, flow across the ductus may be minimal and
color flow echocardiography may not describe it well) or if
findings suggest pulmonary hypertension.
Management
• For a PDA with a shunt large enough to cause symptoms of HF
or pulmonary hypertension, closure should be done after
medical stabilization.
• For a persistent PDA without HF or pulmonary hypertension,
closure can be done electively any time after 1 yr
• Treatment options:
A) Prostaglandin synthesis inhibitor therapy
(eg, indomethacin, ibuprofen)
• It's especially effective in premature infants and is usually
ineffevtive in full term infants.
• Three doses of indomethacin are given IV q 12 to 24 h based on
urine output; doses are withheld if urine output is < 0.6
mL/kg/h. An alternative is ibuprofen 10 mg/kg PO followed by 2
doses of 5 mg/kg at 24-h intervals
• Fluid restriction may facilitate ductal closure
• In premature infants without respiratory compromise, a PDA is
23
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110
generally not treated.

B) Transcatheter occlusion devices
• Transcatheter closure has become the treatment of choice in
children > 1 yr
• A variety of catheter-delivered occlusion devices are available
(eg, coils, Amplatzer ®duct occluder)
C) Surgical repair
• In infants < 1 yr or who have certain anatomic varieties of the
ductus, surgical division and ligation may be preferred over the
transcatheter approach.
• Surgical Complications include phrenic nerve injury, recurrent
laryngeal nerve injury, or chylothorax
D) VATS ligation.
required only for the first 6 mo after closure or if there is a
residual defect adjacent to a transcatheter-placed device or
surgical material.
Aortopulmonary Septal Defect (AP Window )

Definition
It's an opening between ascending aorta and pulmonary artery
with separate aortic and pulmonary valves
Pathophysiology
• It is caused by incomplete formation of the septum in the
developing truncus arteriosus. This results in an abnormal
communication between the aorta and the pulmonary artery
distal to their semilunar valves.
• It's a high flow left to right shunt resulting in rapidly
progressive CHF and early development of high PVR and
Eisenmenger syndrome
• Richardson Classification:
Ø type I is proximal defect, they begin in/near the sinus of
Valsalva and end before the origin of the RPA
Ø type II is more distal defect close to the right pulmonary
artery; frequently extend to the origin of the RPA
Ø type III is actually anomalous origin of either pulmonary
artery from the ascending aorta.
• Associated defects include interrupted aortic arch, anomalous
origin of right or left coronary artery, VSD, PDA, aortic
atresia, or tetralogy of Fallot
• Symptoms and signs of congestive heart failure arepresent.
• Pulses are bounding, and the pulse pressure is wide.
• Because of pulmonary hypertension, the murmur is rarely
continuous.
• The pulmonary second sound is usually split, and the
pulmonary component is loud.
• Pulmonary oedema may be present and may be the cause of
cyanosis
Diagnosis
• Chest X-ray shows cardiomegaly and plethoric lung fields
• ECG shows biventricular hypertrophy and right axis deviation
in the majority of patients.
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111
• Echocardiography can established the diagnosis, confirm

presence of two separate arterial valves
• Magnetic resonance imaging (MRI) is also useful,
• Cardiac catheterization may be required for assessing
pulmonary arteriolar resistance in older patients
Management
Medical treatment
• Heart failure
• PG in cases of associated aortic arch obstruction
Surgical patch closure of defect with correction of associated
defects.
• Surgical correction may be complicated by PA stenosis.
Atrioventricular Septal Defect (AVSD)

Definition
• The terms AVSD, AV canal defect, and endocardial cushion
defect can be used interchangeably to describe this group of
defects
• Deficiency or absence of septal tissue immediately above
and/or below the normal level of the AV valves. The AV valves
are abnormal
Pathophysiology
• The basic morphology of AVSD includes a large, central defect
that may lie above the AV valve or may extend to variable
degrees above and below the AV valve.
• There is a common AV valve annulus that stretches across
both ventricles. There may be a common superior leaflet, or
the superior leaflet may be separated at its distal margin into
right and left components.
• The AV valve may be misaligned with respect to the
ventricles, in association with hypoplasia of the right or left
ventricle.
• The left AV valve is a trileaflet valve made of superior and
inferior bridging leaflets separated by a mural leaflet. There
may be abnormal lateral rotation of the posteromedial
papillary muscle.
• “Wedge” position of aorta (between TV and MV) is absent.
However, aortic valve is elevated and deviated anteriorly;
LVOT is narrowed, but rarely hemodynamically significant,
which gives what is called “gooseneck” LVOT in angiogram
• Most complete AVSDs are in Down syndrome patients (more
than 75%). Most partial AVSDs occur in non-Down syndrome
patients
• Conduction syestem
i) A-V node displaced inferiorly (between CS and
ventricular crest-nodal triangle)
ii) His bundle
a) Travels anteriorly and superiorly from A-V node,
reaching ventricular crest where it fuses posteriorly with
25
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112
common A-V valve annulus

b) Passes along crest of septum, giving off left bundle
branches, then becomes RBB, runs toward muscle of
Lancisi
• Associated Lesions
Pulmonary stenosis, Tetralogy of Fallot, completely
unroofed coronary sinus with left SVC, other conotruncal
anomalies, and heterotaxy syndromes.
• Morphology:
A) Complete AV septal defect
• Consists of a large ostium primum atrial septal defect (ASD)
in the anteroinferior aspect of the septum, a nonrestrictive
inlet VSD, and a common AV valve orifice.
• Rastelli classification (based upon anatomy of anterior
bridging leaflet)
Ø Type A: Common leaflet but cordae attached to crest of
interventricular septum
Ø Type B: Papillary muscle attached to left part of leaflet
from right of septum - rare
Ø Type C: Free floating common anterior leaflet without
cordal attachment to ventricular crest
• A left-to-right shunt occurs at the atrial and ventricular levels
and is often large; AV valve regurgitation may be significant,
sometimes causing a direct left ventricle-to-right atrial shunt.
• These abnormalities result in enlargement of all 4 cardiac
chambers.
• Hemodynamic findings are similar to those of a large VSD.
Over time, the increase in pulmonary blood flow, pulmonary
artery pressure, and pulmonary vascular resistance may lead
to reversal of shunt direction with cyanosis and Eisenmenger
syndrome (see Eisenmenger Syndrome).
B) Transitional AV septal defect
• Consists of an ostium primum ASD; a restrictive inlet
VSD, which may be small or moderate in size; and a
common AV valve.
• The shunt at the atrial level is usually large. The shunt at
the ventricular level is smaller than in complete AVSD,
and right ventricular pressure is lower than left
ventricular pressure.
• The hemodynamics depend largely on the size of the VSD
and whether there is significant AV valve regurgitation.
C) Partial AV septal defect
• Consists of an ostium primum ASD and partitioning of the
common AV valve into 2 separate AV orifices, resulting in
a so-called cleft in the mitral valve (left AV orifice). The
ventricular septum is intact.
• Hemodynamic abnormalities are similar to those of
ostium secundum ASD (eg, left-to-right shunt at the
atrial level, enlarged right heart chambers, increased
pulmonary blood flow) with the additional finding of
variable degrees of left AV valve regurgitation.
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113
• Large left-to-right shunt, as in CAVSD, causes signs of heart
failure (HF—eg, tachypnea, dyspnea with feeding, poor
weight gain, diaphoresis) by age 4 to 6 wk.
• Pulmonary vascular obstructive disease (Eisenmenger
syndrome) is usually a late complication but may occur
earlier, especially in children with Down syndrome.
• Smaller left to right shunts, as in PAVSD, are asymptomatic
unless associated with severe mitral regurgitation. However,
symptoms (eg, exercise intolerance, fatigue, palpitations)
may develop during adolescence or early adulthood.
• Infants with moderate or severe mitral regurgitation often
have signs of HF.
• Large left-to-right shunt shows an active precordium; a
single, loud 2nd heart sound; a grade 3 to 4/6 systolic
murmur; and sometimes a diastolic murmur at the apex and
low left sternal border.
• Small to moderate shunts have wide splitting of the S 2 and a
midsystolic (eg, ejection systolic) murmur audible at the
upper left sternal border. A mid-diastolic rumble may be
present at the lower left sternal border when the atrial shunt
is large.
• A cleft in the left AV valve results in a blowing apical systolic
murmur of mitral regurgitation.
Diagnosis
• Chest x-ray shows cardiomegaly with right atrial
enlargement, biventricular enlargement, a prominent main
pulmonary artery segment, and increased pulmonary vascular
markings.
• ECG shows a superiorly directed QRS axis (eg, left axis
deviation or northwest axis), frequent 1st-degree AV block,
left or right ventricular hypertrophy or both, and occasional
right atrial enlargement and right bundle branch block
• Echocardiography establishes the diagnosis
• Cardiac catheterization is not usually necessary unless
hemodynamics must be further characterized before surgical
repair (for example, to assess pulmonary vascular resistance
in a patient presenting at an older age).
Management
A) Medical
Antifailure therapy (eg, diuretics, digoxin, ACE inhibitors)
before surgery
B) Surgical repair
• Repair should be done by age 2 to 4 months because most
infants have HF and failure to thrive.
• Repair should be done before 6 months, even if infants are
growing well without significant symptoms, to prevent
development of pulmonary vascular disease, especially in
infants with Down syndrome.
• For asymptomatic patients with a partial defect, elective
surgery is done at age 1 to 3 years
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114
• In patients with 2 adequately sized ventricles and no

additional defects, the large central defect (combination of
the primum ASD and inlet VSD) is closed and the common AV
valve is reconstructed into 2 separate valves.
• Surgical complications include complete heart block (3%),
residual VSD, and/or left AV valve regurgitation. Late
complications may include left AV valve regurgitation and/or
stenosis, and LVOT obstruction with or without AR.
• Pulmonary artery banding is no longer recommended unless
associated abnormalities make complete repair in a small
infant high risk.
required only for the first 6 mo after repair or if there is a
residual defect adjacent to a surgical patch.
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115
FIGURE 4−56. Algorithm for the management of AVSD (partial, transitional, and
complete) .
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115
DEFINITIONS:
AVSD = lacks AV jct, but may s9ll
have 2 AV valve orifices
AVSDà AV vlvs lack a figure of 8
shape, and instead are an oval, so
vidence ree Atrioventricular Septal Defect no place for Ao to be wedged, thus
causing the gooseneck deformity.R2
AV Valve a*ached AV Valve a*ached Free Floa9ng

to Ventric Septum to Atrial Septum AV Valve
(Par9al AV Canal) -‐ oZen not classified as AVSD,
similar to an inlet VSD

aka Os&um Primum ASD
Intermediate AVC = Free

Floa9ng with 2 orifices
Transi;onal AVC** = Free
Two Ventricle Orifice Single Ventricle Orifice
Floa9ng w very small VSD and
2 separate annuli (exists per
van Praagh but not Anderson) See VSD Algorithm,
though some evidence
here may apply
Balanced AVSD Balanced AVSD+TOF RV Dominant AVSD LV Dominant AVSD
ASx in Infancy Sx in Infancy May need diure9cs if Sx, but all must May present with cyanosis LV may be able to grow post-‐op, as less -‐Most have tri-‐21, thus incr PVR
be repaired surgically soon. instead of CHF IVS impression into LV; a*aching patch (up airway obst, etc) so poss poor
Down’s may incr risk for phtn, but to R aspect of IVS may allow more
Treat like an ASD. Can be elec9vely Treat early. More likely to have small L
mixed info (see Down’s box) room for LV to growR16
Fontan candidateR14
repaired as a child (2-‐4yo). No Tx sided structures.
needed otherwise.
NHx TOF Surgical RiskR11, R14
-‐Most will get CHF bc of -‐0-‐11% mortality, Long Term PxR15
+Sx Primum ASD NHxR1 incr Qp, w subsequent down fr 29-‐40% in past Σpts 87% survival at
ASx Primum ASD NHxR1 -‐most are Asx, in 1 pulm vasc obst dz that BT Shunt/Sano & Late Repair
-‐Some prefer to place shunt for Qp and 10yrs vs 100% if Nl
-‐of kids ini9ally ASx, 6% Medical Tx series, 10% had CHF Sx would make them a poor
then repair RVOT and AVSD at 2-‐4yo-‐ ? RV
got Sx (SVT, pna, FTT, -‐if symptoma9c and needed earlier candidate for repair later allows for be*er vlv repair and larger
cyanosis) by the 9me of -‐Diure9cs repair. These pts are on.R1 conduit if coronary anom, others rec
surgery at 2-‐4yo more likely to have early repair bc cyanosis, BTS distorts Possibly Revivable
small L sided structures PA’s, PSà AVR R1, R14; -‐must ensure Irreparable Ventricle
Ventricle
or higher PVR, or be patch contoured to avoid resid RVOTOR11
Assoc xx Downs
-‐c/s w Down’s bc phtn makes SV repair less likely to c/s if sev ventric hypoplasia, straddling valves
-‐AV vlv regurg; LVOTO (3%R4) Long Term PxR14 succeed not dividable, Ao stenosis, hypoplas9c
-‐CHF w incr Qp & phtn if Sx early -‐Reop in 40%, avg transverse ArchR11
PA Band Indica;ons 2yrs out for RVOTO
SurgeryR1, R3 -‐c/s PAB if the pt is +Sx, but prefer to RV Dom: c/s ifR11, R16 **very limited dataset/pt #:
PS, LAVVR Pre-‐Op AssessmentR1, R2, Mav, Lai -‐MV Area >4.75cm2/m2, LV inflow dimension >25mm,
Pre-‐Op Ques;ons go straight to complete repair. Some -‐Echo
Elec9vely close at 2-‐4yo in ASx pts Mav apex-‐base LV:RV > 0.8, Ao annulus >6mm (based on
Echo advocate for PAB if <6mo or <5kg [ ] ASD & VSD size & shun9ng cri9cal AS w small LV)
Early repair if +Sx.
[ ] ASD size and unresponsive to Rx, though most [ ] check LVOTO/anatomy (inlet-‐outlet Ln diff) -‐& suffic valvar 9ssue, no ventric non-‐compac9on, and
If signif LA regurg, small L structures [ ] check valve anatomy, cord a*chmt, # paps
[ ] Valve anatomy & cord a*achement say do complete repair if +FTTR1 good LV cavity size w LVEDV >20mL/m2 but re LVEDV
(CoAo, abNl MV, SubAS) then c/s earlier (Rastelli type); #orifices (espec @ L AV vlv) it might be that it is under-‐filled bc of RV vol OD w IVS
[ ] AV vlv regurg repair. Single stage repair is as good or [ ] check amt of regurg pressed into the LV
[ ] #pap muscles (single worse bc if you be*er than mul9-‐stage for L sided xx Biventricular Repair Indica;ons [ ] degree of ventric balance/hypoplasia
Long Term Px R1
close the cleZ, it mayàparachute MV -‐rec complete repair at 2.5-‐6mo or sooner [ ] check for phtn-‐ TR jet, PIG at VSD for RVP LV Dom-‐ c/s if:
(CoAo, Inter Arch, etc)R3 -‐17% need late reop
and obstruct inflowR3 if FTTMav, R1, R13 [ ] check assoc xx -‐AV vlv index (RV vlv area:LV) is >0.5 & RV:LV Ln > 0.66
within next 20 yrs-‐ -‐best at 3mo; as neonate may have more ( ) Parachute MV
[ ] LVOTO/r/o subAo stenosis bc no successful outcome @<this in 1 study, though
-‐Goal to close ASD & restore L AV vlv for L or R AV regurg, problems w AV regurg, PR &ventric fxR9, R13 -‐closing the cleZ w parMV mayàobst vlv RV:LV Ln isn’t validated to measure RV sizeR15
[ ] Degree of phtn competence, without injuring L AV stenosis, resid -‐Surgical Goals:R1 orifice or cause LVOTO, so if signif regurg w the
conduc9on system ASD/VSD -‐Close ASD & VSD cleZ, c/s vlv replacement
-‐Make 2 separate competent vlvs ( )TOF (see above) – 6% of AVSD
Surgical Risk
-‐Reconstruct L AV vlv as bileaflet, though ( ) DORV 6%
-‐2-‐3% in hospital mortality R1, R4 ( ) TGA 3%; ( ) PDA 10%, ( ) LSVC 3%
-‐8.6% for infants, 0.6% for older pts; older reports a 2 patch repair keeps it as trileaflet.
much higher infant mortality ~20%R4 Some c/s the L ant leaflt not w cleZ but w ( )SubAo Stenosis 1% in preop pd-‐ usually a
a commissure fibromusc membrane that can be resected; if a
-‐R/F= CHF, cyanosis, FTT, <4yo, mod+
postop membrane c/s an uncorrected inlet
L AV regurg VSD…, might need future Konno…
-‐in 1 study of 180 pts, 2 of the 3 dead pts were -‐AV nd, His bundle course
infants w sev CHF preop, 3rd died of sepsisR4

-‐bradyarrhyth-‐ SA dysfx, heart blockR1
-‐transient atrial arrhyth 3% & HB 2%R4 Single Patch Two Patch Modified Single
Technique Technique Patch Technique
Long Term PxR1, R3, R4 = 1 patch & divide =sandwich AV vlvs =1 patch w no
Recruit A?empt w Watch & Wait/A?empt Single Ventricle
-‐98% 10 yr survival, w need to repair as infant a R/F for deathR4 AV leaflets bn 2 patches division of AV leaflts Biventric Repair to Recruit Ventricle Pathway
-‐~10% needed reopera9on within 10 yrs of surgery, half were ini9ally -‐RV Cavity recruitment -‐PAB if needed -‐PAB if LV dom or
repaired as infants-‐ reop for subAS or L AV vlv regurg (only 1 vlv was Surgical Risks (all repair types) a*empts – c/s release -‐Pulsa9le Glenn Norwood if RV
replaced)R4 -‐1.6-‐2.8% mortalityR9, R4, R10 septal pap muscle to -‐Glenn
-‐literature range for vlv reop is 7-‐20%R4 1 Patch: Surgical Risk & OutcomeMav free up R AV vlv -‐Fontan
-‐GI problems 15%R6
-‐Age & Preop mod-‐sev AV regurg predicted worse outcomeR4 -‐pleural effusion 14%R6 apparatusR11
-‐3% death at Boston (1970-‐80s) -‐c/s AS fenestra9on if
R/F for long term xx-‐ resid intraop AV regurg, sev dysplas9c L AV vlv, fail to -‐6mo f/u total 26% mod-‐sev L AV vlv
Up to 15% at Venderbilt AV vlv index is <0.5
close the ant (septal) leaflet regurg, no diff what Rastelli type
-‐L AV regurg 6-‐9%, HB w pacer 2-‐4%, (RV:LV vlv size)
-‐AV regurg (mul;-‐ctr study)R6
LVOTO 1%, resid defect 0-‐5%
-‐L AV vlv Stenosis Surgical risk d/o:R1, Mav
-‐Simple, oZen need to divide the vlv
-‐subAo Stenosis -‐Age-‐ best if <6mo bc <phtn
for exposureà decr vlv fx, slightly
-‐late LVOTO-‐ more common w par9al vs complete AVSD, likely bc unlike w -‐AV regurg severity – In recent
higher risk of resid VSD
complete repair, a standard repair doesn’t modify the elongated and mul9center study: LAV vlv regurg at

possibly narrowed LVOT 6mo f/u d/o age at surg, but not LV Dom Risk if BV RepairR11, R15
-‐Repair via transAo rsx of membrane, or patch enlarge the LVOTO via AVSD type (par9al/transit/complete) RV Dom Risk if BV RepairR11 -‐3/32 pts died postop-‐ all w RV:LV vlv area
transAo or RV approach (modified Konno) 2 Patch: Surgical Risk & OutcomeMav, R12 or whether annuloplasty was done (in -‐15/26 had BV repair, rest had Norwood. <0.43. 1/6 pts died aZer SV repair w PAB
-‐residual/recurrent ASD study it was done more in pts w R/F to get SV repair was large VSDm in 3 (1 died, 1 no Glenn bc phtn, 1 Glenn
-‐1990s data-‐ 7% death, reoperate 8%, HB w pacer 3%, LVOTO transverse arch hypoplasia w PDA depend but no Fontan); 3/29 remaining needed
worse preop regurg)R6 Q, narrow L AV vlv, AS, AR
-‐may need valve replacement if severely dysplas9c L AV vlv 2%, resid defect 3% -‐Pre-‐op fxl class early reop bc RV too small ( AS fenest,
-‐Dacron/PTFE makes aneurysm less likely than w pericardium, BTS, or Glenn) all had AV vlv index <0.5
-‐but no reliable way to incr L AV annulus, may put prosthe9c valve in LA -‐Down’s has be*er outcome bc more -‐AS fenest’n helped pt tolerate
above the na9ve vlv less resid VSD, less subAS, less dehiscence of patch redundant vlv 9ssue so be*er repair
-‐but hemolysis w residual ASD bc of Dacron patch, more HB bc w less regurg post
-‐Pa9ents who were Sx as infant were more likely to need reopera9on patch less malleable than pericardium so more torsion at -‐CleZ closure-‐ closing ità less R1In the past, they would all get
(LVOTO, AV regurg/replacement) and to die in the postopera9ve period, suture line reopera9on for regurg
usually assoc w more complex anatomyR3 **?close the cleZ or not-‐ most reop occurred bc of regurg w an Fontan, but recent success if AV vlv
-‐One study 30 pts: Repair as an adult (avg 42yrs) had excellent outcome, w unclosed cleZ, so Mav recs closing it index >0.5 (but also fenestrate
**Transi;onal AVSD Pre/Post theatrial septum
no surgical death, 15% 5yr mortality fr stroke,MI, hep failure, renal failure, -‐TAVSD had highest prevalence
sepsis; reopera9on in 2 pts (6%) for resid ASD or L AV regurg; most pts ASxR5 Mod 1 Patch: Surgical Risk & OutcomeMav, R12, R13 -‐LV may look to small to work pre-‐op
of pre-‐op mod-‐sev L AV regurg bc of underfilling (bc Q via ASD to R
(aka Australian/Nunn technique);
à don’t need to divide AV vlvs, and don’t entrap cords bc patch not sewn to IVS, safe 44%, vs 21% in CAVSD and 32% side) and IVS bows into LV bc of RV vol

Reopera;on of L AV vlvR11 in small infants <5kg; 2 patch might be be*er if VSD>10mmR11 in PAVSD Also had the most OD

-‐3-‐18% of pts have post-‐op L AV regurg needing re-‐op -‐1990s Sydney data-‐ 2.8% death, no signif resid VSD, but trivial VSD in 20% (no reop postop regurg at 33%, vs 22% -‐Things that portend a be*er outcome
-‐can be worse with par9al AVSD vs con9nuous; more likely if cleZ not needed), L AV vlv fx mild regurg in 29%, mod in 5%, no early LVOTO by 3yr f/u. for CAVSD and 29% for w 2 ventric repair
Minneapolis data-‐ no mortality over 10yrs of surgery, similar outcomes as 2 patch PAVSDR6
repaired ini9allyR8 otherwise -‐restric9ve VSD, antegrade Q thru
-‐c/s annuloplasty, cord replacement, or leaflet edge to edge technique -‐maybe be*er for SV vlv competency outcome, and be*er for smaller infants and Asc Ao, DA closed or only LàR shunt,
-‐If regurg is bc ant valve 9ssue is insufficient, can extend it w pericardial neonates w CHF bc easier. w similar UE & LE SaO2
patch w good results -‐Mav-‐ if Rastelli A do mod single, if C then do 2 patch
-‐if need to replace the vlv, some put it above the na9ve vlv, others elevate à Be*er rate of late reop for L AV regurg 2% vs 7% w 2ptch, 10% w 1ptch
the post-‐sup annulus w a dacron patch cut out fr a conduit along the place à -‐recent rev (mul9-‐ctr)-‐ less LAVR-‐ 0% w Mod1patch, 14% w 1patch, 30% w 2patch,
but Mod1patch was done in younger pts, may be confounded…R13
of the prosthe9c vlv
-‐4% L AV vlv replacement mortality, decr fr 30% b4 1990s, & long term Cle_* Closure & Annuloplasty
survival is bn 50-‐65% at 10yrs, w ½ needing subsequent replacement within -‐CleZ closure is acknowledged to be worthwhileR9, 1 stdyR8: Down’s Syndrome & AVSDR1
10 yrs 2% reop vs 22% w/o closure (and incr in vlv regurg in 9% vs -‐more likely to have Complete AVSD; & TOF
28% w/o closure), but closed cleZs were done more recent -‐less likely to have situs or splenic xx, less FTT pre-‐op
-‐Ques9on remains whether annuloplasty at repair is
-‐less likely to have LVOTO, LV hypoplasia, CoAo, other VSDs
worthwhile to prevent L AV vlv regurg
-‐recent mul9center trial found no improvement, and -‐may have higher PVR fr sleep apnea, hypovent, or
maybe worsening outcome for wt gainR6 nasopharyng obst causeing higher CO2
-‐Recent animal model showed reducing annular size in -‐higher PVR:SVR, but improves w 100% FiO2, so hypoxia/
addi9on to cleZ repair àsignif improvement in valve fx; hypercapnea impoves at cath
recs closing cleZ 2/3 to prevent regurg and also not cause -‐11% Down’s do get fixed elevated PVR by <1yo!
stenosis, along w undersizing the annulus to prevent/limit
-‐Surgical outcome for Down’s is same/be*r as gen pop (94%
future dila9onR7
*Per Anderson & others, it’s not a cleZ, bc closing it does vs 86% survival-‐ rev of 206pts, but more unbal w Nl pts R10.
R13)
not à Nl vlv, rather it is a zone of apposi9on bn 2 leaflets
03 04
Acyanotic Cyanotic
w L®RS PBF
02 05
Acyanotic Cyanotic
w/o Shunt ¯PBF
R. Side
01 06
Acyanotic Cyanotic
w/o Shunt PAH
L. Side
ezzeldinmostafa.com
116

Dr. Ezzeldin Mostafa emostafa@med.asu.edu.eg 5. Cyanotic w éPBF
5 Cyanotic with Increased Pulmonary Blood Flow

(Admixture Lesions)
Table of Contents Persistent Truncus Arteriosus (PTA) ................... 11

Total Anomalous Pulmonary Venous Return
5 Cyanotic with Increased Pulmonary
&Coretriatriatum .................................................. 14
Blood Flow (Admixture Lesions) .................2 Anomalies of the Systemic Venous Return ......... 15
Transposition of the great arteries (TGA) .............. 5
“Corrected" TGA (CCTGA) .................................. 11
116
117


(CHD)
terminology
om/pedscards-
Timeline
TGA
• In TGA, the aorta arises from the RV and the PA arises from the
LV, resulting in independent pulmonary and systemic circulations.
• TGA is incompatible with life unless mixing of the circulations
occurs through an atrial and/or ventricular septal opening, or a
patent ductus.
• Severe cyanosis occurs within hours of birth, followed rapidly by
metabolic acidosis; there are no murmurs unless other anomalies
are present.
• Relieve cyanosis by giving PGE 1 infusion to keep the ductus
arteriosus open and sometimes by using a balloon catheter to
enlarge the foramen ovale.
• Surgical repair is the definitive treatment during the early neonatal
life.
CCTGA
• CCTGA is a very different problem to TGA.The main abnormality is
reversed morphology of the RV and LV, but there is
atrioventricular discordance, i.e., the RA connects with the LV and
the LA connects with the RV. The pulmonary and systemic
circulations are therefore in series.
• In the long-term the RV is unable to sustain a normal cardiac
output against systemic vascular resistance and RV failure occurs
in the third or fourth decade. Associated lesions may pose
additional problems
• Patients usually present late in their first decade with exercise
intolerance. Bradycardia caused by complete heart block may be
present.
3
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118

• ECG may show AV block. Echocardiography describes and

delineate anatomy
• Management is controversial: PA banding early in life may
preserve morphological LV function, restore septal symmetry and
reduce systemic AV valve (tricusid) regurgitation. Results of the
double switch or Senning-Rastelli operation to “correct” the
anatomy are best in the first decade of life.
PTA
• In PTA, the primitive truncus does not divide into the pulmonary
artery and aorta, resulting in a single large arterial trunk that
overlies a large ventricular septal defect (VSD).
• Different types are distinguished based on the origin of the
pulmonary arteries and associated defects.
• Patients present with mild cyanosis, significant pulmonary
overcirculation, and heart failure (HF); a grade 2 to 4/6 systolic
murmur is audible along the left sternal border and a mid-diastolic
mitral flow murmur may be audible at the apex.
• Treat HF with diuretics, digoxin, and ACE inhibitors; prostaglandin
infusion is not beneficial except if associated with interrupted
aortic arch.
• Do surgical repair early; one or more revisions are usually needed
as children grow.
TAPVC
• In TAPVC, the pulmonary veins do not connect to the left atrium.
They converge to form a confluence behind the pericardial sac that
connects to the systemic venous system in three ways; supra
cardiac, cardiac, or infracardiac.
• In cortriatrium, the pulmonary veins collect in an intrapericardial
chamber lies superior and posterior to the LA, from which it is
separated by a fibromuscular diaphragm.
• Pathphysiology and clinical presentation depends on the presence
and degree of obstruction in front of the pulmonary venous flow.
• Echocardiography is diagnosic
• Surgical repair is mandatory for all cases and emergency in
obstructed cases.
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119

Transposition of the great arteries (TGA)

Definition
TG) accounts for 5 to 7% of CHD. TGA is AV concordance with
ventriculoarterial discordance. As such, d-TGA implies that the aorta
arises rightward and anterior to the PA and arises from the systemic
RV.
Pathophysiology
• Systemic and pulmonary circulations are completely separated.
After returning to the right heart, desaturated systemic venous
blood is pumped into the systemic circulation without being
oxygenated in the lungs; oxygenated blood entering the left
heart goes back to the lungs rather than to the rest of the body.
This anomaly is not compatible with life unless desaturated and
oxygenated blood can mix through openings at one or more
levels (eg, atrial, ventricular, or great artery level).
• In the early postnatal period, the thickness of the left ventricular
wall is similar to that of the right left ventricle. In the absence of
an associated large ventricular septal defect, the growth rate of
the right ventricle decreases and within a few weeks after birth
the thickness of the left ventricular wall is comparable to that of
the right ventricular wall in a normal heart
• Associated Lesions
Ø Coarctation of the aorta in approximately 5%.
Ø VSD, which occurs in up to 45% of cases,
Ø LVOT obstruction in approximately 25% of cases, due to:
1. Displacement of the ventricular septum into the left
ventricular outflow tract due to increased systemic systolic
pressure in the RV
2. Less commonly, related to a fibrous shelf below the
pulmonary valve
3. Occasionally, abnormal attachment of the mitral valve may
encroach on the outflow tract of the left ventricle
4. The pulmonary valve orifice itself may be small and
sometimes the valve is stenotic.
5. When transposition is associated with a VSD, subvalvar
pulmonary stenosis may be due to displacement of the
outlet septum into the LVOT
• Coronary Arterial Anatomy
1. The description of the coronary artery anatomy in TGA is
dependent upon correct anatomical description of the aorta
relative to the pulmonary artery
2. Enumerative system:
a. based on the observation that the coronary arteries arise
from the sinuses that face the pulmonary root. the observer is
positioned in the nonfacing sinus looking toward the pulmonary
artery
b. the sinus on the right is called sinus 1 and the sinus on the
left is called sinus 2
c. the three main coronaries are considered separately- RCA
(R), LAD(L) and Cx
d. the pattern of branching are then specified by assigning each
coronary to its sinus starting with sinus 1 and enumerating all
three in a counterclockwise fashion
f. 70% have the 1LCx- 2R pattern, 14% have the 1L-2CxR
pattern
g. when the aorta is either directly anterior, right anterior, or
left anterior, the most common type is 1LCx- 2R pattern and
5
119
120

when the aorta is side-by-side the PA the relatively rarer types

of branching are almost always found
• Coronary Ostia
1. in most cases the orifice of the coronary artery is situated
approximately in the middle of the sinus of Valsalva just below
the sinutubular junction
2. minor deviations from this central position is found frequently
3. in approximately 10% cases the orifice of a coronary artery
will arise close to one of the valve commissures
4. ostia can also arise above the commissures in the tubular
portion of the aorta
5. occasionally two ostia will arise from the same sinus (usually
sinus 2)
6. sometimes a coronary artery will take an intramural course,
usually courses between the aorta and pulmonary artery, most
commonly involves the LAD, the proximal aspect of the coronary
artery is completely embedded in the wall of the aorta
• Five groups are considered.
1. Aortopulmonary transposition with inadequate communication
between the pulmonary and systemic circulation.
2. Aortopulmonary transposition with a large communication
between the pulmonary and systemic circulation:
Ø Ventricular septal defect
Ø Atrial septal defect.
3. Aortopulmonary transposition with ventricular septal defect
and pulmonary stenosis.
and hypoplastic right ventricle.
and tricuspid atresia
• Severe cyanosis occurs within hours of birth, followed rapidly by
metabolic acidosis secondary to poor tissue oxygenation.
• Patients with a large VSD, a patent ductus arteriosus, or both are
less cyanotic, but symptoms and signs of heart failure (eg,
tachypnea, dyspnea, tachycardia, diaphoresis, inability to gain
weight) may develop during the first weeks of life.
• Physical examination is rather unremarkable. Heart murmurs may
be absent unless associated anomalies are present. The 2nd heart
sound (S 2 ) is single and loud.
• Neurological complications may manifest with a variety of
symptoms. In infancy, the most common presentation is
drowsiness, often with convulsions, which may be focal.
Hemiplegia often develops within a few hours. The symptoms and
signs that should make one seriously consider the diagnosis of
cerebral abscess are unexplained fever, change in behavior, such
as drowsiness, irritability, failure to recognize the parents, and
convulsions
Diagnosis
• Chest x-ray: the cardiac shadow may have the classic egg-on-a-
string appearance with a narrow upper mediastinum
• ECG shows right ventricular hypertrophy but may be normal for a
neonate.
• Echocardiography: confirms the diagnosis, assess presence of
associated lesions, such as the atrial communication, ductus
arteriosus, ventricular septal defect, and outflow tract stenosis of
120
121

the left ventricle; origin and course of the coronary arteries; and
patterns of blood flow
• Cardiac catheterization is not usually necessary for diagnosis but
may be done for balloon atrial septostomy.
Management Massin MM Midterm results of

A) Medical the neonatal arterial switch operation.
• Prostaglandin E 1 (PGE 1 ) infusion A review. J Cardiovasc Surg (Torino)
Unless arterial O 2 saturation is only mildly decreased and the atrial 1999 Aug;40(4):517-22
communication is adequate, a PGE 1infusion (0.01 to 0.1 Planche C, Lacour-Gayet F, Serraf
mcg/kg/min IV) may help by opening and maintaining patency of A Arterial switch.Pediatr Cardiol 1998
the ductus arteriosus; to increase pulmonary blood flow, which may Jul-Aug;19(4):297-307
Karl TR, Cochrane A, Brizard CP
promote left-to-right atrial shunting, leading to improved systemic
Arterial switch operation. Surgical
oxygenation. However, if the PFO has a small opening, PGE 1 may
solutions to complex problems. Tex
have the opposite effect because the increased blood return to the Heart Inst J 1997;24(4):322-33
left atrium may close the flap of the foramen ovale, leading Losay J, Hougen TJ Treatment of
to decreased mixing. transposition of the great arteries. Curr
• Metabolic acidosis is treated with NaHCO3. Opin Cardiol 1997 Jan;12(1):84-90
• Pulmonary edema and respiratory failure may require
mechanical ventilatory support. Sources for further reading
B) Balloon atrial septostomy (Rashkind procedure) Textbook Chapters

For severely hypoxemic neonates who do not immediately respond Chapter 39: Complete Transposition of
to PGE 1or who have a very restrictive foramen ovale, cardiac the Great Arteries. Cardiac Surgery
catheterization and balloon atrial septostomy can immediately (Kirklin and Barratt-Boyes), 2nd ed.,
improve systemic arterial O2 saturation. A balloon-tipped catheter is 1384-1467.
advanced into the left atrium through the patent foramen ovale.
The balloon is inflated with diluted radiopaque dye and abruptly
withdrawn to the right atrium to enlarge the opening in the atrial
septum. As an alternative to taking the neonate to the
catheterization laboratory, the septostomy procedure can be done
at the bedside under echocardiographic guidance.
C) Surgical repair (arterial switch operation)

• Definitive repair is the arterial switch (Jatene) operation, typically
done during the first week of life. The proximal portions of the
great arteries are transected, the coronary arteries are
transplanted to the native pulmonary root, which will become the
neoaortic root, and the aorta is connected to the left ventricle and
the pulmonary artery is connected to the right ventricle. Survival
rate after surgery is > 95%.
• precise documentation of the coronary anatomy is critical to the
performance of the arterial switch operation
• when the LV to RV pressure ratio is <0.6, these patients can
undergo pulmonary artery banding and systemic to pulmonary
artery shunt placement in order to condition the left ventricle prior
to the arterial switch procedure
• An associated VSD should be closed at the time of primary repair
unless it is small and hemodynamically insignificant.
• Pulmonic stenosis is problematic unless it is mild and can be
addressed surgically at the time of the arterial switch procedure.
• MBT shunt or rastelli operation for TGA,VSD,PS
• Univentricular repair in cases of hypoplastic RV.
• Endocarditis prophylaxis is recommended preoperatively but is
required only for the first 6 mo after repair unless there is a
residual defect adjacent to a surgical patch or prosthetic material.
121
122
Complete TGA
Congenital Heart=Surgery
D-TGA Spring 2016
Path Epi
RVà Aoà Body 5% of CHD, 3:1 M:F
LVà PAàLungs
- D= dextro, classically that NHx
Ao is ant & to right of PA, vs à hypoxia, acidosis, CHFà death as newborn
L (when it is to L of PA) @<6mo (90%)
-fibrous continuity bn pulm -+IVS w/o VSDà worst Sx, but best
vlv & mitral vlv improvement w Rashkind AS
(instead of bn aortic vlv & -+VSDà least cyanosis, but will get CHF &
mitral vlv) & +subAo conus Pulm vasc obst dz (bc RV flow to LV to pulm
èserial circuits. bed), by 3-4moà so need surgery @ <4mo.
-Mix (& survive) d/p ASD, Similar Px if big PDA.
VSD, PDA, but ½ only have -VSD + PSà survival w/o surgery bc PS
a PFO or small PDA protects pulm bed, so less pulm htn. Bigger
-5% have LV outflow tract risk p surg.
obst (= subpulmonary EKG
stenosis). Dynamic (20%)- -RAD (+90-200), RVH earlyà upright V1 T
bc H&P
IV septum bows into LV wave at DOL3; +/-RAH too
bc -cyanosis
of RV pressure (bc it’s at -Biventric hypertrophy if +VSD, PDA, pulm
since birth (100%)
systemic pressures). obst dz, bc of LVH in addition to RVH
-CHF, dyspnea, feeding difficult since birth
Anatomic (Fixed) (rare)-
subpulm stenosis, abNl CXR
PE-
mitral chordal attachment. -egg on a string heart ß narrow superior
-cyanosis, esp in large boys
-VSD- 40%. These pts often (unless +CHF) mediastinum (see the pole like mediast)
-tachypneic w/o retractions
have COA,loud
-single interrupted
S2, w/o Ao murmur if IV septum is intact,
Arch, pulm atresia, ECHO
+murmur if VSD- early/holosystolic & less cyanotic
overriding AVif vlv. -parasternal LAà GA from post LV,
+murmur PS/LVOT obst-soft midsystolic
sharply angles postly to lungs (= PA),
-CHFà hepatomegaly, dyspnea
instead of Nl intertwining of the 2 GA,
Lab-
running parallel w Ao proximally.
+hypoxemia w no change w O2
-parasternal SAà the Nl “circle & sausage”
+ acidosis
is lost, instead u see double circles, w PA
gluc, Ca
in center of heart w/o coronaries coming
RxTx from it. Ao is ant/right to PA w CA’s from
-ABG for pH, correct met acidosis, Ca, & gluc it.
-PGE1 to reopen DA (continue thru surgery) -apical/subcostal àPA from LV, Ao from
-O2 if severe O2%à PVR, PBF (bld RV
flow)à sys art O2 sat -subcostalà check for atrial
-Cardiac cathà Rashkind = balloon arterial communication/shunt w Dopplar
septostomy (thru PFO)à 10% O2sat
-digox/diuretics for CHF
122
123

ascending aorta is brought behind the

artery (PA; shaded) is also transected
made at the proper position in the PA

Triangular buttons of similar size are
the aorta with triangular buttons. B,
PA and is connected to the proximal

coronary arteries are removed from
trunk. C, The coronary arteries are

coronary ostia, and the pulmonary
ß Arterial switch operation. A,
ascending aorta is lifted, and both
transplanted to the PA trunk. The
triangular defects in the proximal

PA, to form a neoaorta. D, The
transected slightly above the
at about the same level. The

The aorta (AO; unshaded) is
SxTx
Definitive Repair
-Senning or Mustardà atrial levelà intra-atrial repair (rarely done)
-can use prosthetic (M), or an atrial septal flap & RA free wall (S) to redirect venous flow to
LA so it goes to LV then PA.
-Rastellià ventricular level (poor longterm Px)
-if +VSD, severe PS, can do LVà intraaventric tunnel à Ao valveà Ao, & do
RVàhomograft/valved conduitàPA. Usually done @ >1yo. 10-30% Mortality
-Arterial Switchà GA level (preferred)
-transfer CA’s to PA, cut GA & connect proximal end to distal end of the other artery
-less long term xx than S&M or Rastelli, only 6% operative mortality
-rare xx = CA obstà MI, supravalve PS, neoartic valvar regurg or stenosis
-LV must be able to support systemic circ, so LV P must be near systemic at time of surg, so
do it shortly after birth (<3wk old), & CA’s must be able to be switched to to PA
-Damus-Kaye-Stansel + Rastelli- if +VSD & subAo stenosis
-Two Stage Switch- if LV P is low (bc u missed the early pd)à PA binding to LV P for a week to
months to attain LVP >85% of RVP. (rapid approach of 1 weekà less scarring)
The Rastelli operation. A, The

pulmonary artery (PA) is divided
from the left ventricle (LV), and
the cardiac end is oversewn
(arrow). B, An intracardiac tunnel
(arrow) is placed between the
large ventricular septal defect
and the aorta (AO) so that the LV
communicates with the aorta. C,
The right ventricle (RV) is
connected to the divided PA by a
123
124

FIGURE 5−11. Algorithm for the management of TGA
10
124
125
H&P
Complete TGA = D-TGA Epi -cyanosis since birth (100%)
5% of CHD, 3:1 M:F -CHF, dyspnea, feeding difficult since birth
PE-
Path -cyanosis, esp in large boys
RVà Aoà Body -tachypneic w/o retractions (unless +CHF)
LVà PAàLungs -single loud S2, w/o murmur if IV septum is intact,
- D= dextro, classically that Ao is ant & to right of PA, vs L +murmur if VSD- early/holosystolic & less cyanotic
(when it is to L of PA) +murmur if PS/LVOT obst-soft midsystolic
-fibrous continuity bn pulm vlv & mitral vlv -CHFà hepatomegaly, dyspnea
(instead of bn aortic vlv & mitral vlv) & +subAo conus Lab-
èserial circuits. +hypoxemia w no change w O2
-Mix (& survive) d/p ASD, VSD, PDA, but ½ only have a PFO + acidosis
or small PDA ↓gluc, ↓Ca
-5% have LV outflow tract obst (= subpulmonary stenosis).
Dynamic (20%)- bc IV septum bows into LV bc of RV pressure
(bc it’s at systemic pressures). Anatomic (Fixed) (rare)- subpulm
NHx
stenosis, abNl mitral chordal attachment.
à ↑hypoxia, acidosis, CHFà death as newborn @<6mo (90%)
-VSD- 40%. These pts often have COA, interrupted Ao Arch,
-+IVS w/o VSDà worst Sx, but best improvement w Rashkind AS
pulm atresia, overriding AV vlv.
-+VSDà least cyanosis, but will get CHF & Pulm vasc obst dz (bc
RV flow to LV to pulm bed), by 3-4moà so need surgery @
RxTx <4mo. Similar Px if big PDA.
-ABG for pH, correct met acidosis, ↓Ca, & ↓gluc -VSD + PSà ↑survival w/o surgery bc PS protects pulm bed, so
-PGE1 to reopen DA (continue thru surgery) less pulm htn. Bigger risk p surg.
-O2 if severe ↓O2%à ↓PVR, ↑PBF (bld flow)à↑sys art O2 sat
-Cardiac cathà Rashkind = balloon arterial septostomy (thru
PFO)à 10% ↑O2sat EKG
-digox/diuretics for CHF -RAD (+90-200), RVH earlyà upright V1 T wave at DOL3;
+/-RAH too
-Biventric hypertrophy if +VSD, PDA, pulm obst dz, bc of
aorta is brought behind the PA and is connected
repaired, and the proximal aorta is connected to

(AO; unshaded) is transected slightly above the
to the proximal PA, to form a neoaorta. D, The
The Rastelli operation. A, The

coronary ostia, and the pulmonary artery (PA;
LVH in addition to RVH

of similar size are made at the proper position
the distal portion of the divided PA. Note that

with triangular buttons. B, Triangular buttons
in the PA trunk. C, The coronary arteries are

coronary arteries are removed from the aorta
level. The ascending aorta is lifted, and both
ß Arterial switch operation. A, The aorta
transplanted to the PA trunk. The ascending

shaded) is also transected at about the same
pulmonary artery (PA) is divided from the

triangular defects in the proximal aorta are
left ventricle (LV), and the cardiac end is

CXR
the neo-PA is in front of the neoaorta.
oversewn (arrow). B, An intracardiac

tunnel (arrow) is placed between the large -egg on a string heart ß narrow superior mediastinum (see
ventricular septal defect and the aorta the pole like mediast)
(AO) so that the LV communicates with
the aorta. C, The right ventricle (RV) is
connected to the divided PA by a valved ECHO
conduit or an aortic homograft. -parasternal LAà GA from post LV, sharply angles postly to
lungs (= PA), instead of Nl intertwining of the 2 GA, running
parallel w Ao proximally.
-parasternal SAà the Nl “circle & sausage” is lost, instead u
see double circles, w PA in center of heart w/o coronaries
coming from it. Ao is ant/right to PA w CA’s from it.
-apical/subcostal àPA from LV, Ao from RV
-subcostalà check for atrial communication/shunt w
Dopplar
SxTx
Definitive Repair
-Senning or Mustardà atrial levelà intra-atrial repair (rarely done)
-can use prosthetic (M), or an atrial septal flap & RA free wall (S) to redirect venous flow to LA so it
goes to LV then PA.
-Rastellià ventricular level (poor longterm Px)
-if +VSD, severe PS, can do LVà intraaventric tunnel à Ao valveà Ao, & do
RVàhomograft/valved conduitàPA. Usually done @ >1yo. 10-30% Mortality
-Arterial Switchà GA level (preferred)
-transfer CA’s to PA, cut GA & connect proximal end to distal end of the other artery
-less long term xx than S&M or Rastelli, only 6% operative mortality
-rare xx = CA obstà MI, supravalve PS, neoartic valvar regurg or stenosis
-LV must be able to support systemic circ, so LV P must be near systemic at time of surg, so do it
shortly after birth (<3wk old), & CA’s must be able to be switched to to PA
-Damus-Kaye-Stansel + Rastelli- if +VSD & subAo stenosis
-Two Stage Switch- if LV P is low (bc u missed the early pd)à PA binding to ↑LV P for a week to months to
attain LVP >85% of RVP. (rapid approach of 1 weekà less scarring)
125
126

“Corrected" TGA (CCTGA)

Definition
CCTGA is a rare CHD (About 0.5%–1% of all babies born with heart
defects) in which there is ventriculo-arterial and atrioventricular. It is
also called L-TGA.
Pathophysiology
L-TGA is a rare form of CHD (less than 1%). Both atrio-ventricular
and ventricular arterial relationships are reversed, i.e. the right
atrium is connected to a morphologic LV that is located on the right
and the left atrium is connected to a morphologic RV located on the
left (ventricular inversion). The great arteries are also transposed, i.e.
the aorta is connected to the morphologic RV and the pulmonary
artery is connected to the morphologic LV. The aortic valve is anterior
and to the left of the pulmonary valve. Hemodynamically, both
systemic and pulmonary circulations are separated and no cyanosis
result. L-TGA is commonly associated with other CHD such as VSD
(50%), PS, TR, AV block other arrhythmias.
• usually asymptomatic unless have other CHD.
• CHF may develop later as the systemic RV fails to pump against
high systemic vascular resistance.
• Signs and symptoms of associated CHD may be present.
• O/E, the S2 is loud and single (the aorta is anterior to the PA).
• A holosystolic murmur at the LLSB may indicate TR (systemic AV
valve).
Diagnosis
• EKG shows absent Q waves in left precordial leads and their
presence in V4R (due to reversed septal depolarization), AVB,
WPW or other arrhythmias may be present.
• CXR shows a straight left upper cardiac border formed by the
ascending aorta.
• Segmental anatomy can be shown by ECHO. Other associated CHD
can also be detected.
Management
A) Medical:
• Management of CHF and arrhythmias if present.
• SBE prophylaxis and strenuous sports restrictions are indicated.
B) Surgical:
• Repair of associated CHD (VSD, PS, TVR).
• Pacemaker implantation may be needed for AVB.
• Double switch procedure (arterial and atrial) has been tried to
treat the failing RV.
Persistent Truncus Arteriosus (PTA)

Definition
Persistent truncus arteriosus (PTA) occurs when, during fetal
development, the primitive truncus does not divide into the
pulmonary artery and aorta, resulting in a single, large, arterial trunk
that overlies a large, malaligned, perimembranous ventricular septal
defect.
It accounts for 1 to 2% of congenital heart anomalies. About 35% of
patients have 22q11 deletion syndrome, which includes DiGeorge
syndrome and velocardiofacial syndromes.
Pathophysiology
11
126
127

A) Morphology
• There are several classification systems in use.
1. The first classification, by Collett and Edwards, is
Ø Type I: The main pulmonary artery arises from the truncus
and then divides into the right and left pulmonary arteries.
Ø Type II: The right and left pulmonary arteries arise separately
(but adjacent to each other) from the posterior aspect of the
truncus.
Ø Type III: The right and left pulmonary arteries arise from the
lateral aspects of the truncal root reasonably distant from each
other.
Ø Type IV: Both pulmonary arteries are supplied by collateral
vessels from the descending aorta. (Type IV is now reclassified
as tetralogy of Fallot with pulmonary atresia.)
2. An updated classification by Van Praagh consists of type A
(truncus arteriosus with ventricular septal defect [VSD]) and
the very rare type B (truncus arteriosus without VSD). Type A
is subdivided into 4 types:
Ø Type A1: The main pulmonary artery arises from the truncus
and then divides into right and left pulmonary arteries.
Ø Type A2: The right and left pulmonary arteries arise
separately from the posterior aspect of the truncus.
Ø Type A3: One lung is supplied by a pulmonary artery branch
that arises from the truncus and the other lung (usually the
left) is supplied by a ductus-like collateral artery
Ø Type A4: The truncus is a large pulmonary artery and the
aortic arch is interrupted or coarctation is present.
• The truncal valve may be tricuspid (60%), bicuspid (25%), or
quadricuspid (10–15%). This valve is usually dysplastic and
manifest with stenosis, insufficiency, or both.
• The VSD is subarterial, with its roof made from the truncal
valve, and its posterior border muscular (80%) or
perimembranous (20%).
• Other anomalies (eg, right aortic arch, interrupted aortic arch
usually type B (between LCCA and LSCA), coronary artery
anomalies (mostly single coronary artery), atrioventricular
septal defect) may be present and may contribute to the high
surgical mortality rate.
B) Hemodynamic consequence
It includes mild cyanosis, significant pulmonary overcirculation
with heart failure and early development of pulmonary vascular
disease.
Infants usually present with mild cyanosis and symptoms and signs of
HF (eg, tachypnea, poor feeding, diaphoresis) in the first few weeks
of life. Physical examination may detect a hyperdynamic precordium,
increased pulse pressure with bounding pulses, a loud and single 2nd
heart sound (S 2 ), and an ejection click. A grade 2 to 4/6 systolic
murmur is audible along the left sternal border. A mid-diastolic mitral
flow murmur may be audible at the apex when pulmonary blood flow
is increased. With truncal valve insufficiency, a high-pitched diastolic
decrescendo murmur is audible over the mid left sternal border.
Diagnosis
• CXR shows varying degrees of cardiomegaly with increased
pulmonary vascular markings, right aortic arch (in about 30%),
and relatively high position of pulmonary arteries
• EKG commonly shows combined ventricular hypertrophy and
evidence of left atrial enlargement.
12
127
128

• ECHO
• Occasionally ANGIO, to delineate associated anomalies before
surgery
• Cardiac MRI, or CT angiography may supplant the need for
catheterization
Management
A) Medical
Treatment of HF (eg, diuretics, digoxin, ACE inhibitors) before
surgery
B) Surgical repair
• Surgical management consists of complete repair. The VSD is
closed so that the left ventricle ejects into the truncal root. A
conduit with or without a valve is placed between the right
ventricle and the confluence of the pulmonary arteries. Surgical
mortality rates have decreased to as low as 10% in recent years.
Because the conduit is placed during early infancy, its size
becomes inadequate as children grow, and the conduit must be
revised during childhood. Branch pulmonary artery stenosis is a
common sequela.
13
128
129
Path
-single arterial trunk w a truncal valve, às pulm, Truncus Arteriosus
Hx
systemic, coronary circ. -cyanosis at birth
-large perimembranous, infundibular VSD just below the
-CHF w/in days/weeks
TA -DOE/feeding, FTT, pulm infections
-4 Types: PBF ↑ in 1, Nl in 2&3, ↓ in 4. (actually, 4 =
TOF)
PE
-coronary art xx w stenotic ostia, abNl branching/diretion -cyanosis & CHF w SOB/↑RR
-Interrupted Ao Arch in 13%, DISTAL TO THE L -bounding pulses, wide PP, w hyperactive precordium,
carotid artery. Q to legs is done thru a PDA displaced PMI laterally
-R Ao arch in 1/3 of these pt
-systolic click at apex & LSB, single S3, harsh 2-4/6
-DiGeorge synd w ↓Ca in 1/3 of these pt regurgitant systolic murmur = VSD at LSB. If ↑PBFà
apical diastolic rumble +/- gallop. If TRà high
pitched, early diastolic, decrescendo murmur
EKG- Nl QRS axis, BVH in 2/3 pt
CXR- cardiomegaly, ↑pulm vssls, R Ao arch in 1/3
Echo
-VSD just below truncal valve (~TOF)
-large, single great artery
The anatomic type of persistent truncus arteriosus (TA) is determined by -no pulm valve seen, only one semilunar valve (truncal valve)
the branching patterns of the pulmonary arteries. A, In type I, the main -check number of sinuses
pulmonary artery (PA) arises from the truncus and then divides into the
-R Ao Arch in 1/3
right (RPA) and left pulmonary artery (LPA) branches. B, In type II, the
pulmonary arteries arise from the posterior aspect of the truncus. C, In type
III, the pulmonary arteries arise from the lateral aspects of the truncus. D, NHx
In type IV, or pseudotruncus arteriosus, arteries arising from the
descending aorta (AO) supply the lungs. . -CHF w/in 2 weeks, 85% die by 1yo w/o Tx; If there is
pulm htn, then by 3-4mo ↓Sx & death in 20s.
RxTx
-CHF- digitalis, diuretics pre-op
-r/o & Tx DiGeorge
-Check Ca, Mg
-irradiated pRBC PRN only, bc pt may have imm dysfx
-ppx for pneumococcus & strep bc of imm defic of T cells
-no live vaccines
SurgTx Operative technique for types II and III truncus arteriosus. A, Two
-Palliative- PA banding to prevent CHF if large PBF, broken lines on the truncal artery indicate the sites of excision of the
but high mortality (1/3) pulmonary arteries (PAs). The vertical broken line is the site of the
-Definitive: right ventriculotomy. A ventricular septal defect (VSD) is under the
-Rastelli at WOL#1 truncal valve (broken circle). B, The VSD is closed with a patch
through a right ventriculotomy (which is visible through the
-close VSD so that LV ejects into the truncus ventriculotomy) in such a way that the truncal artery receives blood
-make sure u know coronary structure first… only from the left ventricle (LV) (LV-to-truncus pathway). The cuff of
-replace Tri valve if signif TR truncal tissue, including the PA orifices, has been excised and
trimmed. C, Continuity of the truncal artery, which is now the aorta
Operative technique for types II and III truncus arteriosus. A, Two broken lines on the
(AO), has been restored with a Dacron graft. The lower end of a
truncal artery indicate the sites of excision of the pulmonary arteries (PAs). The vertical
broken line is the site of the right ventriculotomy. A ventricular septal defect (VSD) is homograft has been anastomosed to the right ventriculotomy, and the
under the truncal valve (broken circle). B, The VSD is closed with a patch through a upper end of the homograft has been anastomosed to the cuff
right ventriculotomy (which is visible through the ventriculotomy) in such a way that containing the PAs.
the truncal artery receives blood only from the left ventricle (LV) (LV-to-truncus
pathway). The cuff of truncal tissue, including the PA orifices, has been excised and
trimmed. C, Continuity of the truncal artery, which is now the aorta (AO), has been
restored with a Dacron graft. The lower end of a homograft has been anastomosed to
the right ventriculotomy, and the upper end of the homograft has been anastomosed to
the cuff containing the PAs
129
130

Total Anomalous Pulmonary Venous Return &Coretriatriatum

Definition
The pulmonary veins do not connect to the left atrium. Instead, the
entire pulmonary venous return enters the systemic venous
circulation through one or more persistent embryologic connections.
It accounts for 1 to 2% of congenital heart anomalies.
Pathophysiology
• The hemodynamic consequence depends on the connection
between the pulmonary venous confluence and the right side of
the circulation. The most common types include:
Ø Return via an ascending left vertical vein that drains to the
innominate vein or SVC (supracardiac TAPVR)
Ø A descending vein that drains infradiaphragmatically to the
portal circulation (infracardiac TAPVR)
Ø Connection of the confluence to the coronary sinus or
directly to RA (cardiac TAPVR)
Ø Mixed type
• The infradiaphragmatic drainage type is invariably severely
obstructed, leading to dramatic pulmonary edema and cyanosis
unresponsive to supplemental O2 . The other 2 types do not
typically involve obstruction and lead to mild signs of heart failure
(HF) and mild cyanosis in the first month of life.
• TAPVR can be accompanied by other complex congenital heart
defects and is frequently associated with asplenia–polysplenia
syndrome (heterotaxy syndrome) with a univentricular heart.
• TAPVR should be differentiated from cortriatrium in which the
pulmonary venous collecting chamber is intrapericardial and lies
superior and posterior to the LA, from which it is separated by a
fibromuscular diaphragm. There may or may not be a
communication between the two chambers; the RA may
communicate with one or both chambers. The true LA contains the
LA appendage and the mitral valve. Pulmonary venous flow
reaches the left side of the heart by passing across the diaphragm
into the LA, and thus is subject to obstruction at this site.
According to the communication between collecting chamber, LA,
and the RA, this may result in hemdynamic sequence range from
like large ASD to like obstructed TAPVR.
Clinical presentation Heinemann MK, Hanley FL, Van
Neonates with obstructed pulmonary venous return present with Praagh S, Fenton KN, Jonas RA,
severe PAH, pulmonary edema, and cyanosis. O/E a parasternal lift Mayer JE Jr, Castaneda AR. Total
and a single, loud 2nd heart sound (S 2 ), with no significant murmur. anomalous pulmonary venous drainage
If pulmonary venous return is not obstructed, symptoms of HF may in newborns with visceral heterotaxy.
be present and physical examination detects a hyperdynamic Annals of Thoracic Surgery 1994
precordium, a loud and split S 2, and a grade 2 to 3/6 systolic 57(1):88-91.
This diverse series of patients
ejection murmur audible along the LSB. A mid-diastolic tricuspid flow
demonstrates that early repair of
murmur may be audible at the lower LSB. Some infants with
TAPVD can be performed without
unobstructed supracardiac or cardiac TAPVR may be asymptomatic.
increasing mortality; however,
Diagnosis shunting may be required in obstructed
• CXR shows a small heart and severe diffuse pulmonary edema TAPVD.
when there is PVO; otherwise, there is cardiomegaly with Lupinetti FM, Kulik TJ, Beekman
épulmonary vascular markings. The typical superior mediastinal RH 3rd, Crowley DC, Bove EL.
shadow of TAPVC to the vertical vein, or the enlarged CS when the Correction of total anomalous
veins drain to this region, which gives the (snowman sign), may be pulmonary venous connection in
helpful in diagnosis in older infants and children than in neonates. infancy. Journal of Thoracic and
• EKG shows RAD, RVH, and occasionally RAE Cardiovascular Surgery 1993
• ECHO is diagnostic. It should delineate the pulmonary venous 106(5):880-5.
anatomy, as well as differentiating between cor triatrium and Another series with low mortality
14
130
131

supramitral membrane, where the membrane lies between the (about 5%) and low reoperative rate
mitral valve and the LAA: in cor triatriatum, the mitral valve and (about 5%). All varieties of TAPVD
the LAA are on the same side of the diaphragm were represented and all patients were
• ANGIO is rarely necessary; repaired under circulatory arrest.
• Cardiac MRI or CT angiography may need to be done to better
delineate the anatomy of pulmonary venous return. Sources for further reading
Management Textbook Chapters
Chapter 35: Total Anomalous
A) Medical
Pulmonary Venous Connection
Treatment of HF (eg, diuretics, digoxin, ACE inhibitors) before Chapter 9: Anomalies of the
surgery Pulmonary Veins. Cardiac Surgery of
B) Surgical repair the Neonate and Infant (Castaneda,
• TAPVR or cortriatriatum with obstruction require emergent surgical Jonas, Mayer and Hanley), 157-166.
repair. In older infants, HF should be treated, followed by surgical Chapter 16: Total Anomalous
repair as soon as the infant is stabilized. Pulmonary Venous Connection
• Surgical repair consists of creating a wide anastomosis between Chapter 17: Cor Triatriatum. Cardiac
the pulmonary venous confluence and the posterior wall of the left Surgery (Kirklin and Barratt-Boyes),
atrium, along with ligation of the vein decompressing the 2nd ed., 645-682.
confluence into the systemic venous circulation. The repair is
different for return to the CS, in which case the CS is unroofed into
the LA and its opening to the RA is closed.
• Surgical repair of cortriatriatum consists of resection of the
membrane with closure of the atrial septum either by direct suture
or with a patch.
Anomalies of the Systemic Venous Return
Pathophysiology
A) Morphology
The anomalies of the systemic venous return are classified according
to the surgical anatomy and the functional disturbance into:
1. Anomalies of systemic venous return to the systemic venous
atrium.
2. Anomalies of systemic venous return to the pulmonary venous
atrium.
3. Anomalies of systemic venous return to both atria.
4. Combination of anomalies of systemic and pulmonary venous
return
1. Anomalous venous return to the systemic venous atrium

a) Drainage of the Left Superior Vena Cava (LSVC) to the Coronary
Sinus
• most common anomaly of systemic venous return, occurring
in 2–4% of all congenital cardiac defects
b) Interrupted Inferior Vena Cava with azygos continuation
frequently associated
• It’s usually associated with left atrial isomerism. The hepatic
veins continue to drain into the right atrium as a single trunk
or as separate vessels
2. Anomalous venous return to the pulmonary venous atrium

15
131
132
Epi- 1% of CHD 4:1 male Total Anomalous Pulmonary Venous Return
Path
-no communication bn PV’s & LA. Instead PV’s drain thru
systemic vns or RA. 4 types:
--Supracaordiac- (½) ßcommon pulm vn sinus drains into R
SVC thru L vertical vn & L innominate vn
--Cardiac (20%)ß common pulm vn sinus drains into coronary
sinus; or pulm vn enter RA separately thru 4 openings Anatomic classification of total anomalous pulmonary venous
--Intracardiac- (20%)ß common pulm vn sinus drains into return. A, Supracardiac. B and C, Cardiac. D, Infracardiac.
portal vn, ductus venosus, hep vn, or IVC. It goes thru
diaphragm at esoph hiatus Surgical
--Mixed type (10%)ß mix of each approaches to
various types of
-Pulm htn 2y bc obstructed vn return (to long a route or bc total anomalous
↑resistance in liver); & pulm vn obst à pulm art htnà pulm vn pulmonary
venous return
congestion, hypoxemia, systemic ↓perfusion
-ASD or PFO is necessary for survival.
-small L side of heart H&P w Pulm Vn Obstruction
Hx- ↑cyanosis, resp distress as neonate, FTT
-↑ cyanosis w feeds, (esp infracardiac) bc common pulm vn
is compressed by the food-filled esophagus
H&P w/o Pulm Vn Obstruction
PE-
Hx
-↑cyanosis, ↑RR w retractions
-CHF w ↓growth, freq pulm infection as infant
-minimal cardiac findings- loud Single S2 & gallop, no
-mild cyanosis since birth
murmur usually but if there it is faint SEM at ULSB
PE
-pulm crackles, hepatomegaly
-↓nourishment, mild cyanosis, CHF Si- ↑RR, SOB, ↑HR,
EKG- RVH w tal R waves in R precordials, +/-RAH
↑liver
CXR- Nl/slightly ↑ heart size, +pulm edema (kerley’s B
-precordial bulge w hyperactive RV impulse. PMI @
lines, diffuse retic pattern), must DDx pna & hyaline mem dz
xyphoid process & LLSB
-quadruple or quintuple rhythem- S2 widely split & fixed,
w accentuated P2. 2-3/6 SEM @ULSB, mid-diast rumble
at LLSB (= inc flow to tricusp vlv) Echo-
EKG- volume overld RVG = rSR’ in V1, +/-RAH -large RV w compressed LV (hypoplastic), large RA &
CXR- cardiogmegaly at RA/RV, ↑pulm vasc markings. +/- mall LA
Snowman sign/figure 8 if supracardiac TAPVR in pt >4mo -interatrial communication (PO 70%, ASD 30%)
-large common chamber = common pulm vn sinus behind LA
NHx
-CHF in both types, w ↓growth, ↑pna’s
SxTx
-2/3 die at <1yo if non-obst type, from a pna (if no surgery)
-Indication/Timing- +obstructionà surgery ASAP as
-Infracardiac type- die p few wks w/o surgery, most die @ <2mo
neonate; no obstruction but +hrt failure @4-6mo
-Goal: reducred pulm vn return to LA
RxTx -Supracardiacà Pulm Vn Sinus to LA & close ASD w
-Tx CHF- digitalis, diuretics if no pulm vn obst patch
-Tx metab acidosis -TAPVR to RAàcut out atrial septum, replace w patch so
-Intubate w PEEP if +severe pulm edema that Q is diverted from vn to LA
-PGE1 to ↑ systemic Q by keeping ductus open if +pulm -TAPVR to Coronary Sinus- connect the sinus to LA by
htn; esp if infracardiac bc it’ll keep ductus venosus open cutting open it’s “roof” (the LA)
too -InfracardiacàPulm Vn sinus to LA.
-Balloon Atrial Septostomy if interatrial comm’n is small
M&M- 5-10% if unobstructed; 20% if infracardiac
-die from post op sudden pulm htn or pulm vn stenosis
-Complications- sudden pulm htn bc L hrt is small/low
complianceà cardiac failure, pulm edema…
-postop arrhythmia
Post op
-no need for SBE ppx unless +obstruction
-may get arrhythmia/SSS & may need pacemaker 132
133

a) left-sided superior vena cava draining into the left atrium

• It’s the most common malformation of this group of
• The orifice of the LSVC is situated in the left upper corner of
the left atrium, between the base of the left atrial appendage
anteriorly and the left pulmonary veins posteriorly.
• The coronary sinus is typically absent, and there may be
(coronary sinus atrial septal defect)
b) Other less common types of anomalies of systemic venous return
to the left atrium include
• drainage of the IVC to the left atrium,
• drainage of hepatic veins to the left atrium,
• hemi-azygos continuation of the left-sided inferior vena cava
to the left-sided superior vena cava, which drains into the left
atrium.
3. Anomalous venous return to both atria

• This group of conditions includes combinations of anomalies
described in the two previous sections.
• An azygos continuation of the inferior vena cava to the right-
sided SVC and of a left-sided SVC to the left atrium is one
example
4. Combined anomalies of systemic and pulmonary venous

connections.
• Numerous variables are present. These anomalies are often
associated with complex cardiac malformations
• Anomalous systemic and pulmonary venous connection to the
right atrium.
ü Left SVC to coronary sinus – right pulmonary vein(s) to right
atrium.
ü Left SVC to coronary sinus – left pulmonary vein(s) to left
SVC.
ü Left SVC to coronary sinus – partial or total anomalous
pulmonary venous to coronary sinus.
ü Azygos continuation of IVC – right pulmonary vein(s) to right
atrium.
• Anomalous systemic venous connection to left atrium and
anomalous pulmonary venous connection.
ü Left SVC to left atrium – right pulmonary vein(s) to right
atrium.
ü Left SVC to left atrium – anomalous pulmonary vein(s) to left
SVC.
ü Anomalous systemic vein (right SVC or hepatic veins) to left
atrium – anomalous pulmonary vein(s) to anomalous systemic
vein.
B) Hemodynamic consequences
• Effect of right to left shunt
• Affect of associated cardiac anomalies
• According to magnitude of the shunt
• Mild desaturation, usually asymptomatic.
• According to associated cardiac anomalies.
Diagnosis
• All investigation modalities will show the findings of associated
cardiac defects
16
133
134

• Echocardiography is diagnostic
• CT angiography and cardiac catheterization are confirmative.
Management
• Anomalous venous return to the systemic venous atrium does not
produce any functional disturbance. The surgical importance lies
in the techniques of venous cannulation for the extracorporeal
circulation
• Regarding drainage of the left-sided superior vena cava to the left
atrium, three surgical procedures have been used:
Ø Ligation of the left-sided superior vena cava;
Ø Reimplantation of the vena cava into the right atrium or
pulmonary artery;
Ø Intra-atrial redirection of flow from the vena cava
• Intracardiac repair of the associated anomalies
17
134
03 04
Acyanotic Cyanotic
w L®RS PBF
02 05
Acyanotic Cyanotic
w/o Shunt ¯PBF
R. Side
01 06
Acyanotic Cyanotic
w/o Shunt PAH
L. Side
ezzeldinmostafa.com
Fundamentals 136 Spring 2016
Dr. Ezzeldin Mostafa emostafa@med.asu.edu.eg Cyanotic w êPBF
5 Cyanotic with ê pulmonary blood flow
Table of Contents Cyanotic Normal Or êPBF Dominant LV,

without PAH ...................................................... 150
5 Cyanotic with ê pulmonary blood flow136
Pulmonary Atresia with intact ventricular
Cyanotic Normal Or êPBF Dominant RV,
septum .............................................................. 150
without PAH ...................................................... 140
Hypoplastic Left heart syndrome (HLHS) .......... 153
Tetralogy of Fallout (TOF)................................. 140
Heterotaxy syndrome ........................................ 155
Absent pulmonary valve (APV) ......................... 143
Univentricular heart ........................................... 161
Double outlet right ventricle (DORV) ................. 146
136

• To review the surgical anatomy of the cardiovascular system Anatomy & Nomenclature
and surgical significance of eachpart. Normal Cardiac Anatomy -
• Structural functional relationships and review anatomy and Anderson Ch2
https://sites.google.com/a/pedscar
• To review the anatomical correlation with the investigations like
the EKG, ECHO (TTE &TEE), ANGIO, and different imaging ds.com/pedscards-com/cardiology-
studies to orient the surgeon’s perioperative management. notes/normal-cardiac-anatomy
• To know my way to understand the embryology of the CVS and Nomenclature- Moss and Adams
its clinic-physiologic classification of the congenital heart
disease (CHD)
ds.com/pedscards-com/cardiology-
terminology
ds.com/pedscards-
Timeline
TOF/PS
• TOF involves a large VSD), RVOTO, and over-riding of the
aorta.
• êPBF, the RVH, and unoxygenated blood enters the aorta via
the VSD.
• Manifestations depend on the degree of right ventricle outflow
obstruction; severely affected neonates have marked cyanosis,
dyspnea with feeding, poor weight gain, and a harsh grade 3
to 5/6 systolic ejection murmur.
• Tet spells are sudden episodes of profound cyanosis and
hypoxia that may be triggered by a fall in O 2 saturation (eg,
during crying, defecating), êPVR(eg, during playing, kicking
legs), or sudden tachycardia or hypovolemia.
• Neonates with severe cyanosis may be given an infusion of
prostaglandin E 1 to open the ductus arteriosus.
• Tet spells is treated by positioning patient in the knee-chest
position and give O 2 ; sometimes,morphine, volume
expansion, NaHCO 3 , propranolol, or phenylephrine may help.
• Surgical Repair is the definitive treatment.
TOF/APV
• APV is defined as total or subtotal absence of pulmonary
leaflets. Stenosis of the pulmonary artery orifice and
aneurismal dilatation of the pulmonary arteries coexist in all
cases.
• It can be associated with simple and complex CV malformation
137
• The most common association is APV and TOF with absence of

ductus arteriosus.
• The main symptoms of APV are PR and bronchial obstruction.
• Early corrective surgery with implantation of a valved conduit
in RVOT and plication of pulmonary arteries or “LeCompte
maneuver” to relieve bronchial compression is indicated in all
patients.
• Persistence of respiratory symptoms require re-evaluation and
if needed re-intervention with the option of implantation
intrabronchial expandable stent.
DORV
• DORV occurs in multiple forms, with variability of great artery
position and size, as well as of ventricular septal defect (VSD)
location.
• There are three main categories; DORV-VSD type, DORV-TOF
type, DORV-TGA type.
• The clinical findings range from mild cyanosis to severe
cyanosis and from like VSD findings to TOF findings.
• ECHO is diagnostic
• Surgical repair is the definitive treatment.
• Biventricular or univentricular approaches are options
depending on several factors: the location of the VSD and its
relationship to the aortic and pulmonary valves, the presence
or absence of PS, and the distribution of the coronary arteries,
size of the RV, and the straddling of A-V valves.
HLHS
• HLHS refers to the abnormal development of the left-sided
cardiac structures, resulting in obstruction to blood flow from
the left ventricular outflow tract.
• Newborn infants with the condition generally are born at full
term and initially appear healthy. As the arterial duct closes,
the systemic perfusion becomes decreased, resulting in
hypoxemia, acidosis, and shock.
• The most useful diagnostic modality is the echocardiogram.
• Differential diagnosis includes other leftsided obstructive
lesions where the systemic circulation is dependent on ductal
flow (critical aortic stenosis, coarctation of the aorta,
interrupted aortic arch).
• Children with the syndrome require surgery as neonates, as
they have duct-dependent systemic circulation.
• Currently, there are three major modalities, primary cardiac
transplantation, a series of staged functionally univentricular
palliations, or hybrid approach. The treatment chosen is
dependent on the preference of the institution, its experience,
and also preference.
UVH
• UVH encompasses not only hearts known as single or common
ventricle, where one dominant and one hypoplastic chamber
are present, and in which one ventricle lacks inlet component
(double inlet or absent AV connection), but also hearts in
which both ventricles possess all the morphological
components but where one ventricle is tiny and unable to
sustain the circulation.
• The physiology and management of UVH depends upon certain
factors:
• Obstruction to systemic or pulmonary outflows.
138
• Obstruction to ventricular inflow and atrial septum and

abnormal systemic or pulmonary venous return.
• Amount of pulmonary blood flow and pulmonary vascular
resistance.
• A-V valve regurgitation
• Definitive management of UVH is staged procedures ending by
TCPC or Fontan procedure.
139
Cyanotic Normal Or êPBF Dominant RV, without PAH
Tetralogy of Fallout (TOF)

Definition Pulmonary stenosis with intact
The tetralogy of Fallout (TOF, Tet) is a congenital ventricula septum (PS-IVS) has
malformation characterized by underdevelopment of the RV been called trilogy of Fallot.
Although PS-IVS is not
infundibulum with anterior and leftward displacement of the
embryologically related to TOF,
infundibula (canal, outlet) septum and its parietal extension (= 4
it does have similar physiology.
words rather than 4 components). The displacement
(malalignment) of the infundibular septum is associated with
RVOTO (stenosis, or in extreme forms, atresia) and a large VSD.
Pathophysiology
Morphology
A. Underdevelopment of the right ventricular infundibulum
results in:
· Anterior-leftward malalignment of the infundibular septum
· This malalignment determines the degree of RVOTO
· A large subaortic ventricular septal defect (malalignment VSD)
results in overriding of the aorta
B. Tetralogy of Fallot with pulmonary stenosis (TOF-PS)
· The hypoplastic RVOT is associated with pulmonary valve
stenosis, which may extend into the pulmonary trunk
· The branch pulmonary arteries usually have normal arborization
and are rarely significantly hypoplastic.
C. Tetralogy of Fallot with pulmonary atresia (TOF-PA)
· Patients have variable pulmonary blood supply:
1) ductus arteriosus
2) aortopulmonary collaterals: prenatal remnant and abnormal
histologic characteristics
· Intracardiac morphology is similar to TOF-PS.
D. Morphologic categories of Right Ventricular Outflow
Obstruction
· Infundibular to valvar stenosis -- most common
· Infundibular + valvar + small annulus -- common
· Isolated infundibular stenosis -- minority
· Dominant valvar stenosis -- rare
· Diffuse right ventricular outflow hypoplasia -- often in infants
presenting with cyanosis
• The VSD is typically large; thus, systolic pressures in the right

and left ventricles (and in the aorta) are the same.
• Pathophysiology depends on the degree of right ventricular
outflow obstruction. A mild obstruction may result in a net
left-to-right shunt through the VSD; a severe obstruction
causes a right-to-left shunt, resulting in low systemic arterial
saturation (cyanosis) that is unresponsive to supplemental
O2 .
• In some children with unrepaired tetralogy of Fallot, most
often those several months up to 2 yr of age, sudden episodes
of profound cyanosis and hypoxia (tet spell) may occur, which
may be lethal. A spell may be triggered by any event that
140
slightly decreases O 2 saturation (eg, crying, defecating) or

that suddenly decreases systemic vascular resistance (eg,
playing, kicking legs when awakening) or by sudden onset of
tachycardia or hypovolemia.
• The mechanism of a tet spell remains uncertain, but several
factors are probably important in causing an increase in right-
to-left shunting and a fall in arterial saturation. Factors include
an increase in right ventricular outflow tract obstruction, an
increase in pulmonary vascular resistance, and/or a decrease
in systemic resistance—a vicious circle caused by the initial
fall in arterial Po2 , which stimulates the respiratory center
and causes hyperpnea and increased adrenergic tone. The
increased circulating catecholamines then stimulate increased
contractility, which increases outflow tract obstruction.
• Associated anomalies include right aortic arch (25%),
abnormal coronary artery anatomy (5%), stenosis of the
pulmonary artery branches, presence of aorticopulmonary
collateral vessels, patent ductus arteriosus, complete
atrioventricular septal defect, atrial septal defect, additional
muscular ventricular septal defects (VSDs), and aortic valve
regurgitation
• Neonates with severe RVOTO (or atresia) have severe
cyanosis and dyspnea with feeding with poor weight gain. But
neonates with mild obstruction may not have cyanosis at rest.
• Tet spells may be precipitated by activity and are
characterized by paroxysms of hyperpnea (rapid and deep
respirations), irritability and prolonged crying, increasing
cyanosis, and decreasing intensity of the heart murmur. The
spells occur most often in young infants; peak incidence is age
2 to 4 mo. A severe spell may lead to limpness, seizures, and
occasionally death. During play, some toddlers may
intermittently squat, a position that increases systemic
vascular resistance and aortic pressure, which decreases
right-to-left ventricular shunting and thus raises arterial
O 2 saturation.
• Polycythemia and clubbing are usual findings in older children.
• Auscultation detects a harsh grade 3 to 5/6 systolic ejection
murmur at the left mid and upper sternal border. The murmur
in tetralogy is always due to the pulmonary stenosis; the VSD
is silent because it is large and has no pressure gradient. The
2nd heart sound (S2) is usually single because the pulmonary
component is markedly reduced. A prominent right ventricular
impulse and a systolic thrill may be present.
Diagnosis Reddy VM, Liddicoat JR,

• CXR shows a boot-shaped heart with a concave main McElhinney DB, Brook MM,
pulmonary artery segment and diminished pulmonary vascular Stanger P, Hanley FL. Routine
markings. A right aortic arch is present in 25%. primary repair of tetralogy of Fallot
• EKG shows right ventricular hypertrophy and may also show in neonates and infants less than
right atrial hypertrophy three months of age. Annals of
• ECHO is diagnostic Thoracic Surgery 1995 60(6
Suppl):S592-6.
• MRI and CT angiography may delineate detailed anatomy
This thirty-patient series had
• ANGIO is rarely needed, unless there is suspicion of a
excellent results: one death, one
coronary anomaly that might affect the surgical approach (eg,
141
LAD arising from the RCA) that cannot be clarified with ECHO. patient requiring balloon
intervention, and the majority with
Management acceptable RV-PA gradients. Age,
A) Medical symptoms, coronary anatomy, and
• For symptomatic neonates with severe cyanosis may be branch pulmonary artery size were
palliated with an infusion of prostaglandin E 1 (0.01 to 0.1 irrelevant to outcomes.
mcg/kg/min IV) to open the ductus arteriosus. Pagani FD, Cheatham JP,
• For tet spells Beekman RH 3rd, Lloyd TR,
Mosca RS, Bove EL. The
Ø Placing infants in a knee-chest position (older children
management of tetralogy of Fallot
usually squat spontaneously and do not develop tet with pulmonary atresia and
spells), diminutive pulmonary arteries.
Ø Establishing a calm environment, and giving O 2 . Journal of Thoracic and
Ø If the spell persists, options for medical therapy (roughly Cardiovascular Surgery 1995
in order of preference) include: 110(5):1521-32.
1. Morphine 0.1 to 0.2 mg/kg IV or IM, This article demonstrates that
2. IV fluids for volume expansion, complete repair of tetralogy can be
3. NaHCO 3 1 mEq/kg IV, accomplished even with extremely
4. propranolol starting at 0.02 to 0.05 mg/kg, titrated small pulmonary arteries and
up to 0.1 to 0.2 mg/kg IV if needed for effect. facilitates pulmonary artery
Ø If persists, systemic BP can be increased with growth. Several patients had
interventional procedures to
ketamine 0.5 to 3 mg/kg IV or 2 to 3 mg/kg IM
stenoses or collaterals.
(ketamine also has a beneficial sedating effect) Di Donato RM, Jonas RA,
or phenylephrine starting at 5 mcg/kg and titrating up to Lang P, Rome JJ, Mayer JE Jr,
20 mcg/kg IV for effect. Ultimately, if the preceding steps Castaneda AR. Neonatal repair of
do not relieve the spell or if the infant is rapidly tetralogy of Fallot with and without
deteriorating, intubation with muscle paralysis and general pulmonary atresia. Journal of
anesthesia may be necessary. Thoracic and Cardiovascular
Ø Propranolol 0.25 to 1 mg/kg PO q 6 h may prevent Surgery 1991 101(1):126-37.
recurrences, but most experts feel that even one Most patients underwent
transannular patching; actuarial
significant spell indicates the need for expeditious surgical
survival at 5 years was 74% and
repair.
freedom from reoperation 76%. All
survivors were doing well with
B) Surgical repair longest follow-up at fifteen years.
• Definitive management Horneffer PJ, Zahka KG,
• Complete repair consists of patch closure of the VSD, Rower SA, Manolio TA, Gott VL,
widening of the right ventricular outflow tract with muscle Reitz BA, Gardner TJ. Long-term
resection and pulmonary valvuloplasty, and a limited patch results of total repair of tetralogy of
across the pulmonic annulus or main pulmonary artery if Fallot in childhood. Annals of
necessary. Thoracic Surgery 1990 50(2):179-
• Surgery is usually done electively at age 3 to 6 mo but can be 83.
done at any time if symptoms are present. This series of 128 patients had
• In some neonates with low birth weight or complex anatomy, reasonable follow-up, some
initial palliation may be preferred to complete repair; the approaching 30 years. The majority
usual procedure is a modified Blalock-Taussig shunt, in which had developed some degree of
the subclavian artery is connected to the ipsilateral pulmonary pulmonary valve insufficiency and
artery with a synthetic graft. most had excellent functional
status.
• Perioperative mortality rate for complete repair is < 5% for
uncomplicated tetralogy of Fallot.
residual defect adjacent to a surgical patch or prosthetic
material.
142
Absent pulmonary valve (APV)

Definition
It’s total or subtotal absence of pulmonary valve leaflets.
Stenosis of PA orifice and aneurysmal dilatation of the
pulmonary arteries coexist in all cases.
Pathophysiology
• The spectrum of the malformation to which APV may be
associated can be categorized into two types:
a) APV with VSD
Ø The most common form with VSD is the association with
TOF with the anterior deviation of the infundibular septum,
malaligned VSD, and the overriding of the aorta. However,
the distinguishing features are unobstructed right ventricular
infundibulum, and aneurysmally dilated pulmonary arteries.
Ø Typically, absent ductus arteriosus is reported.
Ø This type is referred to as Fallot type APV
b) APV without VSD
Ø It’s less frequent form
Ø Muscular VSD may be rarely observed
Ø The association of a patent ductus arteriosus is reported.
Ø It’s referred to as non-Fallot type APV
• The aneurysmal dilatation of the MPA as well as of the right
or let or both pulmonary artery branch(es) may lead to
compression of the major bronchi at the hilum.
• In addition, compression of the intrapulmonary bronchi by
abnormally branhing pulmonary arteries may represent a
serious complication.
• Associated congenital heart defects:
Ø TOF
Ø VSD
Ø ASD
Ø Coarctation of Aorta
Ø Tricuspid atresia
• Patients with APV syndrome are probably best categorized
into two groups, based on the age at presentation:
1. Infants (often neonates) with severe respiratory symptoms
of tachypnoea, air-trapping, cyanosis and wheezing.
Sometimes there is actual respiratory failure. In these
patients the bronchial compression is the dominant
pathology.
2. Older children with less severe symptoms, often those of
tetralogy of Fallot
• The physical signs usually include a pan-systolic murmur at
the left sternal border, together with a variable length
diastolic rumble of pulmonary regurgitation. The pulmonary
second sound is absent.
• In infants particularly, wheezing sounds are common
Diagnosis
• ECG shows signs of RV hypertrophy and there is a right axis
deviation.
• Chest X Ray shows cardiomegaly and hyperinflated lungs
with shift of mediastinum.
143

• Cardiac catheterization is rarely needed for hemodynamic
study.
Management Sources for further reading
• The goals of surgery are:
Textbook Chapters
Ø to correct any intracardiac anomaly,
Ø prevent right-sided heart failure and Chapter 21: Tetralogy of Fallot.
Ø to alleviate or prevent bronchial compression and peripheral Pediatric Cardiac Surgery
lung damage (Mavroudis and Backer), 2nd ed.,
• Conventional treatment of the TOF using a small trans- 276-290.
annular patch may be doomed to failure because of severe
Chapter 13: Tetralogy of Fallot.
pulmonary regurgitation, due to the relatively high
peripheral pulmonary resistances. In such patients, a valved Cardiac Surgery of the Neonate and
conduit should be inserted between the right ventricle and Infant (Castaneda, Jonas, Mayer and
pulmonary arteries. Hanley), 215-234.
• Approaches to Repair the Pulmonary Arteries:
Ø Plication or resection of pulmonary arterial wall.
Ø Resection of central pulmonary arteries.
ØRepositioning of pulmonary arteries anterior to the aorta
144
FIGURE 6−6. Algorithm for the management of TOF-PS and TOF-PA.
145
vidence ree Tetralogy of Fallot
#1 cyanoSc CHD – 3/10,000 births
-‐7-‐1-‐% of CHD
Assoc w tri 13, 18, 21; phenylketonuria,
maternal reSnoic acid intake, mat DM
-‐Alagille synd-‐ JAG1 mutaSon
-‐Digeorge/ chrom 22 microdel
DORV w TOF
TOF + PA TOF
TOF + APV TOF + AVSD Physiology Eval for concurrent disease:1,5
-‐Digoerge (15% of TOF13)
MAPCAS -‐if T cell low (<8k), can get transfusion assoc GVHD unless
irrad the bld
-‐also poss Alagille , Kabuki, CHARGE, VAVTERL or VATER
synd1
-‐forked ribs
NHx5,6 Pre-‐Op Mgt1 -‐scoliosis
-‐d/o degree of RVOTO -‐-‐PGE postnatally if poss PA or ductal dep Qp
-‐10-‐25% dead by 1yo (criScal PS)
-‐40% dead by 3yrs -‐-‐no lasix!
-‐55-‐70% dead by 10yrs -‐?propranolol efficacy to decr spells; no effect
-‐95% dead by 40yrs on peri/post-‐op outcome27 Pre-‐Op Evalua>on1, 5
-‐Die fr: hypoxic spell-‐ 62% (most (80%) by -‐follow for HA, check for polycythemia & poss Echo-‐
3yo), CVA-‐ 17%, brain abscess-‐ 13%6 neuro xx, & brain abscess fr sepSc emboli [ ] check for large VSD + overriding Ao
-‐If relaSve anemia, pt at incr r/o CVA, Tet [ ] check degr of Ao override (r/o DORV if >50%)
spells; check/replete Fe PRN [ ] check for Ao-‐MV conSnuity (r/o DORV)
Propanolol Risks [ ] check VSD Doppler to ensure low veloc mainly RàL Q
-‐hypoglycemia, [ ] check for TV-‐Ao cont = PM VSD (assess conductn sys anatomy); check if it
hypotension, extends to ounlow w absent infundib septum
bronchospasm, hrt block, [ ] check IVS for other VSDs
hrt failure27 [ ] check TR
-‐?blunt post-‐op response to [ ] check for infundib, vlvr, PA stenosis
Pink Tet Cyano>c Tet [ ] check M/Br PA size & conSnuity, ensure antegrade Q thru {A’s; note degr of
NE/E so maybe HD xx?
PS underesSmated if sSlll high PVR in newborn or +PDA
-‐IniSally, we thought must wait Sll 3-‐5yo for -‐if SaO2 75-‐80%à repair2 [ ] check cor art anatomy – 82% Sn, 99% Sp
repair bc of risks(eg HB) of the repair, but it -‐if >6mo, do single stage; if <6mo, debatable d/
was then shown that M&M is worse if repair p o insStuSon; Mavr rec repair by 3-‐6mo2 [ ] check assoc xx 1,5
2yo bc of worsening RV hypoplasia & infundib -‐R Ao Arch – 25%; +/-‐ aberrant ipsi SCA fr DescAo
stenosis & fibrosis, & if repair early, then pulm
annulus grows, unlike w a shunt. ½ get
-‐LSVC 11% -‐ so check for dilated CS
cyanoSc by 6mo and most by 12mo (in a pre -‐LAD fr RCA 5%
echo study). Early (<2yo) repair improved -‐Branch PA xx-‐ 30% (if pt presents as infant)
RVOTO w/o inr in peri-‐op risk, also less phtn fr
-‐MAPCAs-‐ <5% MRI & Cath1
thrombi and less collaterals7
Tet with Coronary -‐ASD-‐ 10% For pre op cor art & PA
Anomaly… -‐PFO 83% anatomy

-‐2-‐5% of TOF has abNl CA anatomy, w a major art over the RV
infundibulum; devision would à MI/death26
-‐No consensus on mgt-‐ can do a conduit, or a tailored R
ventriculotom &patvh, or a TA-‐TP approach…26 Tet Spell Tx5
-‐some feel anom LAD doesn’t preclude transatrial/PA repair12 -‐occurs @ <1y-‐5yo p agitaSon/dehrdraSon

àcalm pt, morph/ketamine, O2, H2O/IVF, inr
SVR w NE/phenyleph, decr met acidosis,
Single Stage Late Repair Single Stage Early Repair Two Stage Late Repair12 knees to chest, ?propranolol
-‐PotenSal Benefits9, 11: -‐PotenSal Benefits:
-‐repair by 6-‐12 months in pink Tets -‐preserve LV fx, exercise capacity, decr risk of late arrhyth by -‐No CPB/circ arrest in the young infant
avoiding ventruculotomy to RV, divide (instead if resecSng) RV -‐No RV ventriculotomy
muscle bundles, be[er cog dvp w/o prolonged cyanosis, avoid -‐Less need for transannular patch (TAP)
tet spells, stop exposing RV to high P so less RVH, preserces -‐PotenSal Risks:
myocardial and electrcl fx, may improv pulm angiogenesis/ -‐Need 2 surgeries
alveoli dvp, avoids shunt thrombi, CHF fr BTS, PA distorSon fr -‐BTS clot/ death before complete repair
BTS, less $$ -‐distort PAsà stenosis (surgeon dependent); long RV P OD
-‐PotenSal Risks: -‐Do if <4kg, do mBTS iniSally (if _Sx) and complete repair at
-‐arrhythmias, CPB run in se_ng of immature organs 6-‐12mo, when pt weighs >4-‐5kg; L sided BTS if poss isolated LPA

RV-‐PA Conduit Ventriculotomy + Transannular Transaor>c + Transpulmonary

-‐Gore-‐Tex, Hancock, Contegra… RVOT Patch
Patch Approach Approach
-‐may need RVOT patch if RVOTO too small, -‐preferred by Fraser12, on CPB w/o
-‐Mavr rec TAP if RV:LV P >0.7
but à later diln/aneurysmal Sssue circ arrest/low flow if poss
aler RVOT rsxn2
-‐RVOT rsxn thru TV, patch close VSD,
-‐Jonas rec TAP if if MPA/annulus
Trans-‐Annular Patch enlarge PV annulus to 2-‐3mm above
> -‐2 to -‐3 Z score below Nl… Nl, enlarge Pas w pericard,+/-‐
-‐may be needed, but incr r/o PT later
monocusp vlv
Exercise Ac>vity15
-‐limit based on pt’s HD status… Outcomes of Early vs. Late Repair in the Current Era
-‐-‐Boston8 1970s (n=57)-‐ all <2yo, avg 8mo at repair; 31 needed trans-‐annular repair (TAP), f/u obtained in 45pts at ~17yrs post: 10 needed re-‐op, main;y for RVOTO.
-‐avoid if exercise induced life-‐threatening RVOTO in 6 pts w/o TAP and 2 w TAP (33 vs 6%, p 0.04); PV replaced in 1pt; 41/45 were NYHA I, and 4 were II; survival 86% at 20yrs; freedom fr re-‐op: 93% at 5yr,
arrhythmias 79% at 20yr. No diff in survival or re-‐op if pt got TAP. Lower wt at opà 2.6x incr in long term mortality; emergent surg, geneScs, sex had no impact; earlier yr of
repair & geneSc synd increased risk of re-‐op. Only 1 pt had VTà ICD placed. Problems w study: many lost to f/u, limited clinical data…
-‐if RVP >1/2 systemic, sev PR, w RV diln
arrhythmiasà rec restrict to low dynamic & -‐-‐Oklahoma9 1971-‐97 (n=294, TOF PS or PA)– 10yr f/u: 68% primary repair, 21% staged repair, but 23% had complex anatomy (eg pulm atresia). à11% hospital
mortality for primary repair, 18% for staged repair, 15% for palliaSve repair (BTS only). Fr 1990-‐1998, 2%, 12%, 0% mortality for primary, staged, palliaSve repair. R/
staSc acSviSes Heart Block2 F-‐ longer circ arrest, PA patch, earlier yr of op, PFO closure. If survived to discharge, then 98% 20yr survival (88% if pulm atresia). 14% needed re-‐op at 20 yrs (57%
-‐<1% (5% in early period) if pulm atresia): no diff if primary or staged repair or of repaired as infant. BUT-‐ avg age was 2yrs, but since 1990 it is 7mo; ½ pts via transatrial approach since 1990
(increased fr prior decades); need more TAP for infants (64 vs 39%); TAP & age at op were NOT signif R/F for mortality; arrhythmia not checked. Claim 28 vs 11%
mortality for staged vs primary repair, but ?selecSon bias-‐ why pts picked for each type (?worse dz w staged pts). Re-‐op for RVOTO in 14%-‐ R/F = complex dz (eg
pulm atresia or need for PA patch plasty) & incr circ arrest Sme, but NOT age at repair, staged vs primary or TAP use.
Survival:13 -‐-‐Mayo10 1955-‐60 -‐21yr f/u of 160 pts who survived past 1st 30 days. Most operated on at >5yo. Survival (actuarial): 5yr-‐ 95%, 20yr 92%, 25yr 87%, 32yr 86%. Having
TOF in Pregnancy13 -‐1yr survival -‐ 97% a prior BTS didn’t affect 30yr survival, but Waterston & Po[s did decr it. No change in 30 yr survival if pt had a TAP. Older age at surg (e.g if >12yo then 76% 30yr
-‐low risk, ~near gen pop if ASx & good hemodynamics
-‐if RVOTO, sev PR, RV dysfx, then incr vol of PG canà LV -‐20 yr survival if alive 1 mo post-‐op: 98% survival vs 93% if <12yo at surg), prev HF, post-‐op RV:LV >0.5 had incr long term mortality. Of the 22 late deaths, 10 were fr sudden death, 3 during late re-‐op, 2
during HF. NYHA: I 77%, II 17%, III 6%-‐ no assoc w BTS use or TAP use. Degree of PR (present in 33% pts) didn’t affect NYHA. 10% needed late re-‐op, w 88% 30 yr
dysfx; higher rate of miscarriage, highest risk if sev PR or RV -‐30yr survival: 90% freedom fr re-‐op; re-‐op was for resid/recur VSD, RVOT false aneurysm, resid MPA stenosis; avg re-‐op 9yrs post iniSal.
syst dysfx22
-‐vaginal deliver rec’d for most pts15 -‐-‐Ro[erdam-‐ 1968-‐1980-‐ 77 surviving pts, 15yr f/u, 22% w mod-‐sev TR, all had sev RV dil’n, sev PR more common if TAP use, 89% exercise capacity worse if worse RV
-‐though recent Eu study showed CV events common: 8% of dil’n or incr RVP-‐ much lower capacity in pts w TAP. 88% freedom from re-‐op. 2 pts had VT>10 beats, 7 had SVT
PG had CV event(mostly SVT), 6% offspring mortality21
-‐-‐Linz, Austria-‐ 1995-‐06-‐ f/u of pts w repair at <4mo (n = 25 <1mo w repair for PDA dep lesion vs 65 pts >1mo <4mo); no BTS used; ulm atresia excluded; most had
ventriculotomy; TAP used in 84% neonates, 58% rest; pf/u Sme 5yrsà no 30 day mortality; 2% late mortality. 8%re-‐op, 2pts for RVPA conduit; No diff in LOS, ICU
Long Term Complica>ons & Response Sme, vent Sme, complicaSons, or re-‐op.
SBE ppx1, 17
-‐Use in cyanoSc pts, pts w palliaSon, and for 6mo -‐-‐Houston12 1990s-‐ (n=144, TOF PS or PA) – 91 single stage (mean at 1yo), 34 2 stage repair (mean 18mo if PS, 15mo if PA; all PA had 2 stage ): 98% had TA/TP repair
post patch repair; or if pt has a prostheSc vlv or a w 68% w mini transannular repair; all pts w anom LAD able to get TA-‐TP repair; 18 has PA repair, 8 (of 32) of which was bc of shunt related stenosis; no circ arrest. 7
conduit of the 11 PA pts got a homogral. 1 stage vs 2: no diff in age or wt at repair, or pre-‐op sats; outcomes-‐ nodiff in extubn Sme, ICU or hosp LOS, or RVOT pk veloc. 2 pts
w BTS got ?shunt related endocardiSs, tx w abx
-‐-‐Ann Arbour14 (n=61) all neonates, 95% 5yr survival, but 24 fad TOF-‐PA, and 6 nonconf PAs; 49 need TAP, 52 w circ arrest, abd only 58% freedom fr reop rate
Dysfx -‐-‐Toronto15 1990s (n=227) – 1 & 2 stage-‐ Mortality 2-‐6%, fell over Sme to 0% w 1 stage repair. If <3mo, à incr ICU/hosp LOS, è ”opSmal Sme is 3-‐6mo”
-‐RV & LV are inSmately related;
dyssynchony in ctrctn is assoc w decr
exercise capacity & ventric arrhyth15 Arrhythmias13, 15, 17, 25
-‐LV syst dysfx in 21% of pts (n=500, but 80% PR & PS in the future13, 15, 19
-‐35 yr f/u-‐ 12% sust VT, sudden death 8.3%15 (0.15%/yr16) -‐mod-‐sev PR occurs in at least 30% pts w TAP or pulm
had TAP), ~30yr f/u, mainly mild20 -‐sudden death 6% over long term (7.5% @30yr f/u17); assoc w ventric vlvotomy17
-‐RV diast dysfx w “sSff RV” , but beneficial if dysfx, resid VSD, resid RVOTO, sev PR -‐worse PRà worse exercise capacity; & if ch à RV dysfx, VT,
+PR bc limit regurg…/limit diln; due to -‐but, risk of sudden death isn't predicted by non-‐sust VT on holter, & sudden death
worse myocard injury at surgery19 no benefit if anS-‐arrhyth Rx
-‐QRS >180ms is related to RV dil, sust VT, & sudden death; decr in QRS
-‐best to assess w MRI; free PR à up to 40% regurg fx, but Pulmonary Valve Replacement13, 15, 19
-‐resync Tx does improve Rvsyst fx & decr it’s not as bad as AR bc insp’n can drive fwd Qp, & bc PVR is -‐all bio vlvs (xenogral, homogral, stented bioprosthesis) are finite in dur’n & 2nd vlv
dur aler RV vol decr p PV change is assoc w decr risk of arrhyth13; QRS low, short distance to lungs, unless +branch PA stenosis, then
QRS dur17 may last < 1st
dur predicts Px, as does duraSon of change (incr >3ms/yr à poor Px)15 àxx. This, balloon stent/plasty can help delay surg. Can
-‐must follow both RV & LV fx by MRI serially -‐mech vlv-‐need more anScoagn…
-‐EP study-‐ if able to induce VT, it predict sust. VT or sudden death follow RV vol serially w MRI, but hard bc not reproducible & ? -‐procedural risk-‐ 1-‐4% mortality, w 86-‐95% 10yr survival, incr if RV dysfx
bc decr fx predicts decr Px17 -‐inducible VT, Sx’ic VT or syncope à c/s ICD What to do w akineSc part -‐freedom fr reop: 5yr 81%, 10yr 58%, 15yr 41%
-‐ICD placed for primary prevenSon (p presync, sync, palpn, nonsust VT, -‐PR is usually ASx unSl aler 30yo, the rapid Sx of RV failure, PerQ PV Replacement15
-‐likely to improve Sx/fxl class, w decr RV size/TR/PR/RV dysfx (syst 1st, then diast latr)17
QRS>180ms, induc VT)à 8%/yr w appropriate shock; ICD for 2y DOE, death, more likely if pt had an ounlow tract patch15 -‐Melody Valve-‐ bovine IJ vlv in a stent
Ao Root Dila>on15 -‐What to do if ASx but >30% PR?
prevenSonà 10%/yr appropriate shock (n=121); 6%/yr had -‐if signif branch PA stenosis in se_ng of PR, must Tx -‐Study of 155 pts, no early, li[le late
-‐can à AR Indica>ons: -‐Compelling: Sx/decr exercise abillity, VT w Sx/syncope, sustained VT on
inappropriate shocks mainly fr SVT or sinus tach16 aggresively15 mortality, w freedom fr reop: 10mo 93%,
Holter, serial RV diln or decr RV EF, incr RV end syst vol, new TR w RV diln or atrial
-‐15-‐87% reported – d/o definiSon/method -‐PV replacement does decr RV size, QRS dur; also decr monomorphic -‐restricSve physiology of RV in adult (not post-‐op) is 2.5yr 68%, 5yr 84%, 5.8yr 70%
arrhyth
of assess… VT inconsistently, so anlaSon/Rx too (cath or surgical) for best results beneficial, w less DOE, decr hrt size, bc it prevents RV diln fr -‐xx = compress cor art if near RVOT (so
-‐Strong: QRS>180ms or serial incr in QRS dur, or need surg for other reason
-‐no agreement on age/degree to repair to decr risk15 PR (but this hasn’t been shown by MR15) check w angio 1st), vlv failure (Tx w a 2nd
-‐Debatable: RV diast vol >170ml/m2, pt request
-‐?if exercise induced ventric arrhyth predicts sudden death, & Melody)
-‐progressive AR, or root diln >55cm is suppression of ventric arrhyth w exercise doesn’t predict Px17
-‐Toronto15, 17: beyond diast RV >170, syst RV>85ml/m2 likely ineffecSve above
-‐PS-‐ usually fr conduit stenosis; c/s balloon diln if RVP> -‐Outcome: 5yr event free survival rate: 76% if pre-‐op QRS >180ms, 90% if <180; if post-‐ -‐not good if RVOT patch was used or very
accepted as Sme to operate -‐ IART & focal atr tach-‐ 98% success rate w abatn, @~4yr f/u18 50%LVP, or if lower if +RV dysfx, signiff diff in L or R lung Qp, op QRS was >180ms, then 71% vs 91% if <180ms (HR 3.7 if >180ms pre, HR 6.8 if QRS dilated RVOT; must be 22mm or less17
-‐similar in nature to Marfan’s Ao rt diln17 -‐MulSctr study, n=556, avg 36yo-‐ 43% w arrhyth-‐ 20% had atria-‐ IART r/ 2y dyspnea; obst is a major problem in pts w a pulm didn’t decr post-‐op); events = death, PR, VT, Sx HF)23
-‐?if b-‐blockers help to prevent f = incr w RA enlarge OR 6.2, htn OR 2.3, a-‐fib r/f older age 1.1, LAD 3.2, homogral or bio vlv, can stent to delay vlv replacement17 -‐Meta-‐analysis (n=595)-‐ early mortality 2%, late mort 0.5%/pt-‐yr, redo-‐PVR 1.9%/
progression17 incr # surgeries OR 1.5; Ventric arrhyth 14.6% r/f = # hrt surg OR 1.3,
pt-‐yr; signif decr RV vol(63ml/m2 in diast, 37 in syst) no diff in pooled mean RV EF or
QRS dur (but OR only 1.02, LV diast dysfx OR 3.); a-‐fib & ventric arrhyth
QRS dur24
much incr p 45yo25
Double outlet right ventricle (DORV)

Definition
DORV is associated with concordant atrio-ventricular connection
but it can occur with or without transposition of the great
arteries. The DORV accounts for 1-1.5% of all congenital heart
diseases
Pathophysiology
A) Morphology
• Pathologists define the DORV when more than 50% of
both great arteries arise from the RV, but surgeons use
the term when 90% of the arteries arise from the right
ventricle.
1. In the DORV, the aorta and the pulmonary artery are
related to the morphologic RV. There are three essential
gross morphologic features that allow the many variants
to be distinguished:
I. The relationship of the great arteries to the ventricles
1. Aorta to the right and posterior to the pulmonary
trunk (normal arrangement present in a 3% of the
RVOT cases).
2. The great arteries side by side with the aorta to the
right of the pulmonary trunk is the usual arrangement
in DORV (approximately 64%). Physiologically similar
to Tetralogy of Fallot.
3. Aorta to the right and anterior to the pulmonary trunk
(24% of the RVOT cases). Physiologically resembles
TGA, specifically D-TGA with VSD.
4. Aorta to the left and anterior to the pulmonary trunk
(rarest arrangement representing a 7% of the RVOT
cases _ L-TGA_ pattern).
II. The relationship of the VSD to the great arteries

1. Provides the morphologic LV with its only outlet
2. Subaortic
3. Subpulmonic
4. Beneath both (doubly committed)
5. Beneath neither (non committed)
III. The presence or absence of outflow obstruction to RV
outflow
1. Pulmonic stenosis occurs in 41-72% of patients with
DORV
2. When pulmonic stenosis occurs, the VSD is almost
always subaortic
3. RV blood is diverted into the aorta with a decrease in
the oxygen saturation
2. The most common combination is the side-by-
side relationship of the great arteries, with the aorta
directly to the right and with the VSD in the subaortic
location.
• The Taussig-Bing anomaly involves the same great vessel
relationship, but there is a subpulmonic VSD
146
(approximately 8% of cases)
B) Hemodynamic consequencs
• The LV blood selectively enters the aorta because of the
proximity of the VSD to the aorta.
• In the absence of pulmonic stenosis, the physiologic
consequences are similar to those of an isolated VSD. but if
there is pulmonic stenosis in addition, physiology
resembles Tetralogy of Fallot
• A low pulmonary vascular resistance PVR permits RV blood to
flow almost exclusively into the pulmonary trunk and permits
a substantial portion of the left ventricularblood to enter the
pulmonary circulation.
• Aortic O2 saturation is normal, and pulmonary artery (PA)
blood flow is increased.
• DORV can be associated with an atrial septal defect (ASD),
anomaly of the coronary arteries, different grades of aortic
stenosis or pulmonic artery stenosis
• The patients’ symptoms vary greatly depending on the
severity of the defect, and associated defects.
• The patient may be either asymptomatic or symptomatic.
• Symptoms include feeding difficulties and as a
consequence poor weight gain, failure to thrive. Other
symptoms include fatigue, difficulty breathing, and cyanosis.
• The findings on physical examination vary significantly
depending upon the presence and location the VSD
and pulmonic stenosis. The findings range from mild cyanosis
to severe cyanosis and from like VSD findings to TOF findings.
Diagnosis
• ECG may show associated conduction defects such as a
prolonged PR interval, first degree AV block. there are signs of
RV hypertrophy and there is a right axis deviation.
• Chest X Ray shows cardiomegaly and increased pulmonary
blood flow if there is VSD with mild or no pulmonary stenosis.
If PS is present, chest radiography shows a normal heart size
and normal-to-decreased pulmonary vascular markings
study.
Management
A) Medical
• In cases of DORV, PS; same as TOF management
• In cases of DORV, no PS, same as VSD management
B) Surgical repair
• Definitive management
• The surgical technique will depend upon the several factors:
the location of the VSD and its relationship to the aortic and
pulmonary valves, the presence or absence of pulmonary
stenosis, and the distribution of the coronary arteries. There
are also factors such as A-V discordance, size of the right
ventricle, and the straddling of A-V valves:
Ø Intraventricular tunnel of VSD to Aorta +/-
transannular patch or extracardiac conduit RV to PA for
PS
147
Ø In Taussig-Bing Heart, Close VSD to PA + arterial

switch procedure
Ø Complex intraventricular tunnel VSD to Aorta with
infundibular resection
Ø Fontan procedure
• Endocarditis prophylaxis is recommended preoperatively
but is required only for the first 6 mo after repair unless
there is a residual defect adjacent to a surgical patch or
prosthetic material.
148
FIGURE 6−20. Algorithm for the management of DORV/VSD ± LVOTO.
149
Cyanotic Normal Or êPBF Dominant LV, without PAH
Pulmonary Atresia with intact ventricular septum

Pathophysiology
1. RV cavity :
• RV cavity size is variable. Most patients have both right ventricular
hypoplasia and hypertrophy, both of which may be severe.
• The size of the tricuspid valve annulus is related to the degree of
cavity hypoplasia. In addition, the tricuspid valve leaflets are often
thickened and chordae are abnormal in number and attachments.
2. Coronary arteries :
• The diminutive and hypertensive RV has persistence of RV
myocardial sinusoidal coronary artery connections.
• The fistulous communications can connect the hypertensive right
ventricle to one or both coronary arteries with the LAD coronary
artery is the commonest.
• The coronary arterial flow is anticipated to occur from the high-
pressure RV into the sinusoids during systole, thus providing blood
with venous oxygen saturations to the coronary circulation.
However, during diastole, the low pressure in the right ventricle
could result in blood flowing preferentially from the coronary
arteries to the RV cavity, rather than perfusing the left ventricular
myocardium. This has been referred to as coronary artery steal
with resultant left ventricular failure.
• Sometimes the proximal connection between the coronary artery
and the aorta is absent, and perfusion of the involved coronary
circulation is retrograde from the right ventricle (right ventricular-
dependent coronary circulation).
• Intimal fibromuscular hyperplasia often occurs in the coronary
arteries linked with the sinusoids and may be responsible for
myocardial ischaemia and infarction, involving either the right or
the left ventricle or both.
• Same as severe critical PS
• In infants with pulmonary atresia, the second sound is not heard or
is soft at the upper left sternal border. Ejection murmurs are not
audible at the upper left sternal border
Management
• The decision between univentricular and biventricular repair
depends on the size of the right ventricular cavity and the presence
or absence of coronary artery fistulae.
• Resuscitative measures may be needed in neonates with severe
cyanosis and few patients may need transcatheter PDA stenting.
1. In cases of well developed RV and mild or no TR

• Transcatheter technique: creation of an opening by both laser and
radiofrequency techniques followed by balloon angioplasty
• Surgical valvotomy/valvectomy +/- transannular patch
• If the obstruction is not adequately relieved, it may be necessary
150
to provide additional pulmonary blood flow through PGE1 infusion,

transcatheter PDA stenting, or establishment of aortopulmonary
shunts.
2. In cases of well developed RV and severe TR
• This combination is frequently associated with Ebstein
malformation of the tricuspid valve and has a high mortality.
• Several approaches have been recommended
Ø Balloon valvuloplasty and PDA closure by stopping PGE1 and
using indomethacin or ibuprofen
Ø If the pulmonary valve cannot be opened sufficiently to
provide adequate pulmonary blood flow, PGE1 infusion could
be continued and, if necessary, an aortopulmonary shunt
could be introduced. However, high pulmonary arterial
pressure may result in severe pulmonary regurgitation,
which could aggravate the tricuspid regurgitation and
precipitate right ventricular failure.
Ø Beyond the age of about 3 months, bidirectional Glenn
procedure can be performed. At a later stage, the tricuspid
valve can be closed and Fontan procedure is completed
3. In cases of hypoplastic RV
• A right ventricular volume of less than 35–40% has been
considered unlikely to sustain total cardiac output, but
because estimates of right ventricular volumes are not very
reliable in infants, tricuspid valve size may be used in
decision-making. Values that have been considered
inadequate include:
Ø Tricuspid valve diameter less than 8 mm in a newborn
infant;
Ø Tricuspid to mitral valve diameter ratio less than 0.7;
Ø Tricuspid valve diameter Z-scores of –3 or smaller
• A systemic-to-pulmonary arterial shunt can be introduced
until 3–4 months of age, at which time a bidirectional
Glenn procedure is performed and completion Fontan at a
later stage.
4. In cases of hypoplastic RV and coronary sinusoids
• In the neonatal period, a systemic-to-pulmonary arterial shunt or
prolonged PGE1 infusion is recommended.
• After the age of 3–4 months, bidirectional Glenn procedure could
be performed.
• Other procedures were described like closing the tricuspid valve,
with thrombo-occlusion of the right ventricular cavity before the
age of 10–12 months and attempts to close the sinusoids and open
the pulmonary outflow. However, these procedures have no
convincing evidence to support the effectiveness and need to be
studied more carefully.
151
FIGURE 6−26. Algorithm for the management of PA/IVS (10 steps, see FIGURE
6−25 ).
152
Epi
<1% CHD; 2.5% of critically ill CHD pt Pulmonary Atresia (with IVS)
Path
-Atresia w diaphragm like membrane (80%) or atretic infundibulum (20%)
-Valve ring & main PA hypoplastic
-PA trunk is usually not atretic, VSD intact (unless it’s TOF)
-RV sizeà survival:
-Tripartite type of PAà inlet, trabecular, & infundibular parts of RV Nl
Schematic diagrams of right
-Bipartite type of PA à no trabecular part of RV ventriculograms that illustrate three types
-Monopartite type of PA à only the inlet is present of pulmonary atresia with intact ventricular
-Coronary Anomalies associated- ↑P in RV decompresses thru the dilated coronary septum. A, Normal right ventricle (RV). B,
microcirculation into LCA & RCA. Sometimes the coronaries are obstructed prox’ly, & get QTripartite type that shows all three portions
from desaturated RV blood only. (inlet, trabecular, and infundibular) of the
RV. C, Bipartite type in which only the
-RV myocardium- can be ischemic, infracted, fibrosed, w endocardial fibroelastosis, poor inlet and infundibular portions are present.
compliance à ↑post op mortality D, Monopartite type in which only the inlet
-ASD/PFO & PDA is needed to survive (to get Q to lungs) portion of the RV is present.
-PDA provides Q to lungs, unless there are Ao collaterals to lungs (rare)
Echo-
-thick, immobile, atretic pulm valve w no Q on dopplar
Hx
-↑cyanosis, ↑RR if distressed -RVH w small cavity
-Single S2 (bc no P vlv…) -small, patent Tri vlv
-RàL ASD shunt
-no murmur, unless soft murmur of TR & soft
continuous murmur of the PDA -PDA runs vertically from Ao Arch to PA
-hepatomegaly if ASD/PFO too small -Tri vlv size correlates to RV cavity size (↑Tri stenosis =
↑small RV)
-coronary artery fistulas on dopplar
EKG- Nl QRS axis (unlike TA), +RAH, +LVH, maybe
RVH.
NHx
-50% death w/in 1 month if not treated, 80% by 6mo – die as
CXR- Nl/large heart bc of ↑RA; ↓pulm vssls markings; PDA closes
concave PA segment
RxTx
-PGE1 to keep PDA open
-cardiac cath & angiography – check coronary anatomy pre-op,
do balloon ASD
-c/s laser assisted pulm valvotomy & balloon pulm valvuloplasty
SurgTx – d/o RV size
-Two Ventricular repair
-1) connect RV to PA w a systemic to PA shunt via a transannular RV outflow patch.
If you do this, don’t do balloon ASD bc u need RV pressure to drive Q forward. OR, do a
transpulmonary valvotom & L sided BT shunt if pulm vlv is good
-2) do cardiac cath 6-18 months later to check if RV size has increased. If O2 sat is
>70%, RV vol increased, & forward Q thru pulm vlv à ↑Px. Then occlude the ASD & see if
pt tolerates this, if so, then he can get a 2 ventricular repair.
-3) à Then close ASD & do RVOT reconstruction & close PDA
-1½ Ventricular Repair- of RV not big enough for 2 ventricles, but too good to abandon
-1)Glenn anastomosis to bring SVC to PA, bypassing RV. Then IVC continues thru
RA/RV normally
- One Ventricular Repair (Fontan) – if pt has monopartite RV or if +coronary fistula
-If Monopartite RVà systemic to PA shunt, no RV outflow patch needed, then a
staged Fontan
-If rudimentary RV (w ↑RV P) & sinusoidal channelsà must decompress RV by
valvotomy so that when you place outflow patch there wont be reversal of coronary flow into
RV (bc it would à ischemia)
-Systemic to PA shunt, then Fontan
Hypoplastic Left heart syndrome (HLHS)

Definition
Hypoplastic left heart syndrome refers to the abnormal
development of the left-sided cardiac structures, resulting in
obstruction to blood flow from the left ventricular outflow tract.
The syndrome includes underdevelopment of the left ventricle,
aorta, and aortic arch, as well as mitral atresia or stenosis
Pathophysiology
• The central anatomical features are:
Ø Aortic valve atresia or hypoplasia with associated marked
hypoplasia or absence of the left ventricle.
Ø The aortic arch and ascending aorta are functionally a branch
of the ductus arteriosus–thoracic aorta continuum, with
retrograde flow through the aortic arch into a diminutive
ascending aorta, which is usually 1.5–3mm in diameter.
Ø The left atrium is small and frequently hypertrophied.
Ø An interatrial communication is common, although a
congenitally small foramen ovale may be present
Ø Mitral stenosis or hypoplasia is present in approximately 60%
of patients, and the remaining 40% have mitral atresia.
• A less common variation includes malalignment to the right of
the common atrio-ventricular canal with regard to the
muscular ventricular septum.
• 10% of patients with HLHS have double-outlet right ventricle
rather than the usual normal relation of the great arteries.
• Cases involving TGA with LV hypoplasia and pulmonary artery
or RV hypoplasia and aorta are not considered to be HLHS.
• Many patients with mitral hypoplasia or stenosis (patent left
ventricular inflow) have prominent endocardial fibroelastosis
with myofibril disarray.
• The left ventricle generally does not form the apex of the
heart. This is a distinguishing key anatomical feature between
HLHS and critical aortic stenosis with a small left ventricle.
• The coronary arteries are normally distributed and supplied by
retrograde flow of blood from the PDA down the hypoplastic
ascending aorta
• Circulation and viability depends on:
Ø Adequate interatrial communication
Ø Patent ductus arteriosus.
Ø Pulmonary vascular resistance
• Depending on the degree of ductal patency:
Ø Newborn infants generally are born at full term, and initially
appear healthy.
Ø Mild cyanosis with tachypnoea and tachycardia
Ø With closure of the arterial duct, the systemic perfusion
becomes decreased, resulting in hypoxemia, acidosis, and
shock.
• Usually, no heart murmur, or a non-specific heart murmur,
may be detected.
• The second heart sound is loud and single because of aortic
atresia.
• Often the liver is enlarged secondary to congestive heart
153
failure
• A small subset of patients have restriction of blood flow from
the left to right atrium because of an inadequate or absent
atrial communication which results in severe left atrial
hypertension, and decreased blood flow into the right atrium
and the dominant right ventricle, resulting in decreased flows
into both the systemic and pulmonary circulations with
subsequent cardiogenic shock and profound cyanosis at birth,
and are likely die in the absence of an intervention
• Peripheral pulses may be normal, diminished or absent.
Diagnosis
• ECG shows RA enlargement and RV hypertrophy.
• Chest x-ray shows cardiomegaly and increased pulmonary
vascular markings.
• Echocardiography is the most useful diagnostic modality
• Cardiac catheterization is only needed if an intervention such
as creation or enlargement of the inter-atrial communication is
required
Management
a) Medical
• The natural decrease in pulmonary vascular resistance shortly
after birth results in a volume shift from the systemic to the
pulmonary circulation, also impairing systemic perfusion.
Satisfactory systemic perfusion and oxygenation can be
maintained in the newborn by ensuring patency of the ductus
arteriosus, maintaining an adequate intravascular volume and
avoiding large differences between systemic and pulmonary
vascular resistances
• A continuous infusion of prostaglandin E1 at a dose of 0.01–
0.05µg/kg/min maintains patency of the ductus arteriosus
• Addition of 1–4% carbon dioxide to the inspired gas mixture or
nitrogen results in an increase in the pulmonary vascular
resistance, favourably influencing the ratio of pulmonary to
systemic flow and promoting adequate systemic perfusion
• A continuous infusion of sodium nitroprusside has been used to
decrease an abnormally elevated systemic vascular resistance,
and the perioperative use of the systemic vasodilator
phenoxybenzamine has gained popularity in some centres
b) Surgical
• Three basic strategies for the neonatal management of HLHS
have evolved over the last 4 decades:
• Staged surgical palliation with a Norwood procedure,
• hybrid palliation with surgical bilateral pulmonary artery
banding and transcatheter ductal stenting,
• orthotopic transplantation.
Norwood Procedure
1) stage I
• The Norwood operation consists of constructing a new aortic
root and arch, disconnecting the pulmonary trunk from the
pulmonary circulation, and incorporating it into the systemic
outflow tract. A modified Blalock-Taussig shunt, or right
ventricle to pulmonary arteries conduit is constructed to supply
blood to the lungs.
154
Epi Hypoplastic Left Heart Syndrome
1% of CHD
Path
-LV hypoplasia w AS or MS & hypoplasia of AsAo & AoArch
-15% w ASD, 10% w VSD, 75% w CoAo
-30% w CNS abnormality- agenesis of corpus callosum, holoprosencephaly,
micrencephaly, etc.
-Prenatal- ↑PVR>SVRà RV maintains perfusion to desc Ao/placenta thru
PDA (RàL) & to prox Ao & coronaries/brain thru retrograde flow. In HLHS,
Postnatal: ↓PVR & close DAà ↓Q Ao & ↓CO àshock & metab acidosis bc
there’s no LV to take over pumping…
-Maintaining Q to body relies on size of PDA & relatively high PVR (so the RV Anteroposterior view of chest film (A) and lateral view
blood doesn’t flow into lungs & instead goes L to systemic circ thru PDA). of an aortogram (B) of a 1-day-old newborn with
-Also need an ASD/PFO bc LA will build up w Q coming from the lungs. Thus hypoplastic left heart syndrome. The heart is enlarged,
and the pulmonary vascularity is increased, with marked
large ASD allows LàR atrial shunt, to prevent pulmonary edema. Here pulmonary venous congestion and pulmonary edema
arteries sat at 80s%. Small ASDà cant get oxygenated blood to RV for (A). The aortogram, obtained with injection of a
radiopaque dye through an umbilical artery catheter,
systemic circ & you get backup à very low O2 sats & pulm edema, which à shows a hypoplastic ascending aorta (thick arrows) with
postnatal death. small coronary arteries (thin arrows) filling retrogradely,
a large patent ductus arteriosus (PDA), and pulmonary
artery (PA) branches.
Sx
-very ill w/in hrs of birthà ↑HR, SOB, crackles, weak pulses, vasoconstriction
at limbs. Gray-blue color +/- cyanosis bc of poor skin Q. NHx
-S2 loud, single (no AoVlv sound, high PVR). No murmur, or a 1-2/6 SEM at -Pulm Edema & CHF @WOL#1, shock,
precordium. ↓%O2, acidosis, death
-CHF- hepatomegally, S3 gallop
-EKG- RVH, +/-large R in V5&6 = dilated RV not LV. Rx Tx
-CXR- pulm vn congestion/pulm edema, +/- big heart -Intubate & ventilate w O2
-ABG- ↓PaO2 slight, Nl PCO2 (still able to kish CO2); severe metab acidosis -Correct metabolic acidosis
>>PCO2 (poor Q to tissues…) bc of ↓CO -PGE1 to reopen PDA
-Echo- small LV, dilated RV, big TriVlv; hypoplasia of Ao/AoAnnulus, -Balloon atrial septostomy to decompress
no/small MV, +/-CoAo; ASD/PFO w LàR shunt LA & improve O2% (temporary fix)
-genetic, ophtho, neuro eval, check
Surg Tx – 3 options: Norwood-Fontan (favored), transplant, or support only intracranial anatomy…
-1) Norwood:
-First Stage Norwood as neonate- ¼ Mortality w noncardiac anomalies &
sev pulm vn obs R/F.
-Cut Main PA. Close the Distal stump with Patch. Ligate the DA.
-With GoreTex, Shunt Q from R subclavian to R PA (= Modified B-T
Shunt) for Q pulm, & to prevent CHF, & prevent pulm htn:
-Attach prox main PA to AscAo to allow RV Q to go to desc Ao/systemic
circ
-Cut atrial septum to allow mixing so that oxygenated Q from lungs will get
to RV for systemic circ.
-2) Bidirectional Glenn Procedure or Hemi-Fontan Schematic diagram of the Norwood procedure. A, The heart with aortic
atresia and a hypoplastic ascending aorta and aortic arch are shown. The
-Bidirectional Glenn = Cavopulmonary shunt bn SVC to R PA (instead of main pulmonary artery (PA) is transected. B, The distal PA is closed with
R subclav, I am using SVC to get Q to R PA for lungs). @3-6mo to reduce a patch. An incision that extends around the aortic arch to the level of the
volume overld to RV, 5% mortality. ductus is made in the ascending aorta. The ductus is ligated. C, A
-Hemi-Fontan- augment central PA w/o dividing SVC. Use intra-atrial modified right Blalock-Taussig shunt is created between the right
subclavian artery and the right PA (RPA) as the sole source of pulmonary
batch to exclude IVC blood from PA blood flow. Instead of the Blalock-Taussig shunt, a homograft conduit
-3) Modified Fontan- 12-18mo; 40%Mortality may be placed between the RV and PA bifurcation as shown (Sano
-Unrestrictive Interatrial Communication modification). By the use of an aortic or pulmonary artery allograft
-Tri Valve Competency (TR = ↓Px of Fontan) (striped area), the main PA is anastomosed to the ascending aorta and the
aortic arch to create a large new arterial trunk.
-PA to Desc AO anastomosis w pressure Hemi-Fontan operation. A, A Blalock-
gradiant <25mmHg (unobstructed) Taussig shunt is taken down (arrow). An
-PAs are undistorted w low PVR incision is made in the superior aspect of A popular modified Fontan operation.
the right atrial appendage (RAA) A, Bidirectional Glenn operation or
-Preserve RV Fx extending it into the superior vena cava superior vena cava (SVC)–to–right
(SVC), and a horizontal incision is made pulmonary artery anastomosis. B,
in the right pulmonary artery (RPA). B, Cavocaval baffle–to– pulmonary artery
The lower margin of the RPA incision and (PA) connection, with or without
the adjacent margin of the incision in the fenestration.
RAA and SVC are connected. C, The
connection is completed using pulmonary
allograft. An intra-atrial patch is placed to
direct SVC blood to the pulmonary
arteries.
• The Norwood procedure with modified Blalock-Taussig shunt.

Ø In the classic Norwood procedure,
Ø Pulmonary blood flow is provided by a Blalock-Taussig
(BT) shunt, which directs blood from the innominate or
subclavian artery to the pulmonary arteries via a
polytetrafluoroethylene tube.
Ø Due to the lower PVR relative to systemic vascular
resistance, there is continuous forward flow into the BT
shunt, not only throughout systole, but also during
diastole. This results in lower systemic diastolic blood
pressure and “coronary steal” that may result in
decreased myocardial perfusion
• The Norwood procedure with RV-to-pulmonary artery conduit.
• Early, it was attempted by Dr. William Norwood then
abandoned in favor of the BT shunt. Decades later, Sano and
several authors have resurrected this technique in an attempt
to address the issue of coronary artery steal.
• The RV-PA has the advantage of eliminating the diastolic runoff
and coronary artery steal but the ventriculotomy adds the risks
of direct myocardial injury and arrhythmias.
• This surgery usually is done within the first 2 weeks of a
baby’s life. Surgeons create a “new” aorta and connect it to
the right ventricle. They also place a tube from either the aorta
or the right ventricle to the vessels supplying the lungs
(pulmonary arteries). Thus, the right ventricle can pump blood
to both the lungs and the rest of the body. This can be a very
challenging surgery. After this procedure, an infant’s skin still
might look bluish because oxygen-rich and oxygen-poor blood
still mix in the heart.
2) Stage II
• Bi-directional Glenn Shunt Procedure
• This usually is performed when an infant is 4 to 6 months of
age.
3) stage III
• Fontan Procedure
• This procedure usually is done sometime during the period
when an infant is 18 months to 3 years of age.
Heterotaxy syndrome
Definition
1. Heterotaxy: "Heteros" means different, "taxis" means

arrangement (from the Greek).
2. Heterotaxy is defined as an abnormality where the internal
thoraco-abdominal organs demonstrate abnormal
arrangement across the left-right axis of the body.
3. Isomerism: "isos" means equal, "meros" means part (from
the Greek).
4. Isomerism in the context of the congenitally malformed heart
is defined as a situation where some paired structures on
opposite sides of the left-right axis of the body are, in
155
morphologic terms, symmetrical mirror images of each other.

5. Heterotaxy accounts for approximately 3% of cases of
congenital heart disease.
156
FIGURE 6−37. HLHS surgical management.
157
Pathophysiology
• Heterotaxy syndrome is a complex syndrome that occurs when
the axes of the body fail to rotate correctly when developing in
the womb.
• This broad term includes patients with a wide variety of very
complex cardiac lesions.
• Patients with heterotaxy can be stratified into the subsets of
asplenia syndrome or heterotaxy with isomerism of the right
atrial appendages (Ivermark syndrome) and polysplenia
syndrome, or the subsets of and heterotaxy with isomerism of
the left atrial appendages
• Paired organs, such as the lungs or kidneys, are often mirror
images of one another instead of having the unique
characteristics of right and left that are normally present.
• There may be levocardia, dextrocardia, or mesocardia (midline
or indeterminate apical orientation).
• When congenital absence of the spleen is diagnosed, there is a
lifelong risk of overwhelming infection.
1. Right isomerism:
• Bilateral structures with morphologic right characteristics,
such as:
Ø Bilateral morphologic right bronchi,
Ø Bilateral trilobed lungs,
Ø Bilateral right atria, atrial appendages exhibiting right
morphologic features.
• The sinus node is usually present bilaterally
• Accompanying heart defects may include:
Ø transposition of the great arteries,
Ø common atrioventricular (AV) valve,
Ø ventricular hypoplasia or single ventricle physiology,
Ø pulmonary atresia and pulmonary vein obstruction.
• There is no left atrium to receive pulmonary venous drainage,
and total anomalous pulmonary venous drainage into a
systemic vein is seen in > 50% of cases.
2. Left isomerism:
• Bilateral structures with morphologic left characteristics, such
as:
Ø bilateral morphologic left bronchi,
Ø bilateral bilobed lungs,
Ø bilateral left atria, atrial appendages exhibiting left
morphologic features,
• The sinus node is usually absent or atretic
• Accompanying heart defects may include:
Ø bilateral superior vena cavae attached to bilateral
morphologic left atria,
Ø The most distinctive and therefore the most diagnostically
useful clinical feature is inferior vena caval interruption with
azygous continuation
Ø Common atrium,
Ø Common atrioventricular valve,
Ø atrioventricular septal defect,
Ø Partial anomalous pulmonary venous connection.
Ø dextrocardia is associated with up to 50% of cases.
158
Ø Fetal complete heart block. Among fetuses with complete

heart block and accompanying structural heart defects, left
atrial isomerism is the most common cause.
• There is a high incidence of abnormalities of the renal tract,
especially biliary atresia
• The prognosis of patients with complex cardiac lesions and
heterotaxy is poor. The 1-year mortality is >85% for patients
with asplenia and >50% for patients with polysplenia.
• A careful evaluation of all systems is mandatory prior to
referral for cardiac surgery. In general, the intracardiac
structures and connections can be accurately defined by
experienced cardiologists.
a) Right isomerism
Ø Almost without an exception, obstruction of the pulmonary
outflow tract, as well as common mixing situations is
present. Thus, cyanosis is by far the most common
presentation
Ø Occasionally present with severe respiratory distress and
cyanosis resulting from an obstructed anomalous
pulmonary venous connection.
Ø Occasionally present with serious extracardiac anomalies
b) Left isomerism
Ø The clinical findings are non-specific and will reflect the
associated lesions.
Ø In contrast to right isomerism, the heart disease may be
relatively mild.
Ø Such patients may never be diagnosed as having
isomerism unless extracardiac anomalies, such as biliary
atresia or intestinal malrotation, draw attention to the
abnormal arrangement of the abdominal organs
Diagnosis
• Chest X Ray allows appreciation of tracheobronchial
anatomy, the position of the cardiac apex, the gastric bubble,
liver and spleen
• ECG shows:
Ø In right isomerism; The P-wave axis is generally normal
because the right sinus node is usually the dominant atrial
pacemaker, alternating QRS patterns (due to antegrade
conduction through either one of the two AV nodes), or
supraventricular tachycardia (attributed to reentry between
paired atrioventricular nodes).
Ø In left isomerism; The ectopic atrial pacemaker (wandering
atrial pacemaker) can shift from one site to another and the
P-wave axis is abnormal (ectopic atrial bradycardia),
Complete atrioventricular block, and atrial fibrillation and
atrial flutter may be seen occasionally
• Echocardiography is diagnostic for the cardiac lesions
• Routine abdominal ultrasound,
Ø To establish the state of the spleen in all patients
Ø Focus on the biliary tree, is warranted even in the absence of
jaundice in infants with left isomerism.
159
= fail to differentiate R & L sided organs
-Symmetric, midline liver Heterotaxia (Splenic Syndromes)
-discordant cardiac apex & stomach bubble
Asplenia -biliary atresia in neonate w CHD
-symmetric main stem bronchi on CXR
Path -superior P axis on EKG
-no spleen
-major organs have bilat Right sidedness – bilat 3 lobed,
symmetrical, midline liver; GI malformations w malrotation;
wrong side stomach Polysplenia
-Cardiac xx- 2 SA nodes. Path
-Nl IVC (unlike in polyspleniaà absent hepatic IVC, uses -multiple spleens
azygos instead) -bilat L sideness
-TGA w PS in 80% à sev newborn cyanosis -2 L lungs, bilat hyparterial bronchi, symmetric liver,
-Single ventrical, common AV valve often no BG, malrotation
-TAPVR to extracardiac structures in most -less PS than in asplenia (13%),
-complete blood mixing bc of cv abNlies -no hepatic segment of IVC so azygos/hemiazygous
-↓PBF bc of Pulm vlv stenosisà cyanosis at birth & CHF makes up diff
-TGA, PS, TAPVR pssole
PE -CHF if ↑PBF; less cyanosis bc less PS
-cyanosis, PS or VSD murmur, midline liver
PE
EKG -lesscyanosis
-ECDà superior QRS axis -CHF
-P axis- Nl, or alternate bn lower L & lower R quadrants bc of
2 sinus nodes EKG
-RVH, LVH, or BVH -ectopic atrial rhythm in most, sup QRS axis bc of
ECD, RVH/LVH, some have complete HB
CXR
-Nl hrt size, ↓pulm vssls CXR
-hrt is at R, L or middle chest -mild-mod CM w ↑pulm vssls, midline liver,
-symmetric liver
-tracheobronch symmetry Lab
-some have Howell Jolly bodies bc of hypofx of
Echo spleen
-check for IVC presence, check for PS
NHx
Lab -less deadly than asplenia (60% mortality at 1y)
-Howell Jolly & Heinz bodies on periph bc no spleen (but can
be Nl) RxTx
-Tx CHF, c/s PA banding
NHx
-95% die at <1yo w/o Tx SurgTx
RxTx -pacer if block
-PGE1 at birth if severe cyanosis -correct as needed…
-PCN ppx bc no spleen
-Immunize againste Hib, mening, pna
SurgTx
-systemic to PA shunt if pulm stenosis ,TAPVR
Structure Asplenia Syndrome Polysplenia Syndrome
Systemic veins Bilateral SVC (65%); single SVC usually right Bilateral SVC (33%); single SVC right or
(35%) left (66%)
[*]
Normal IVC in all, but may be left-sided Interrupted IVC (absent hepatic segment
(35%); (interrupted IVC extremely rare) of IVC) with azygos continuation right or
left (85%)
[†]
IVC and aorta on the same side, either right Juxtaposition of IVC and aorta
or left occasionally
Normal hepatic veins to IVC (75%) Bilateral, common hepatic vein to RA or
LA
[*] [†]
Pulmonary veins TAPVR with extracardiac connection— Normal PV return (50%);
supracardiac or infracardiac—(>80%), often
with PV obstruction Right PVs to right-sided atrium and left
PVs to left-sided atrium (50%) (but not
extracardiac)
Coronary sinus Absent coronary sinus (most) Absent coronary sinus (most)
Atrium and atrial Bilateral right atria (with bilateral sinus node) Bilateral left atria
septum
[†]
Absent atrial septum (common atrium) Single (or common) atrium, primum ASD
common; primum ASD (100%), secundum (60%), or secundum ASD (25%)
ASD (66%)
[*]
AV valve Common (single) AV valve (90%) Normal AV valve (50%); single AV valve
rare
Complete AV canal, usually Partial ECD common (with large primum
defect)
Ventricles and VSD always present VSD frequent but not always.
cardiac apex
[†] [*]
Single ventricle (50%) usually morphologic Two ventricles usually present; VSD
RV or undetermined; two ventricles (50%) (65%); DORV (20%)
DORV (>80%)
Left apex (60%); right apex (40%) Left apex (60%); right apex (40%)
[†]
Semilunar valves Stenosis (40%) or atresia (40%) of Normal pulmonary valve (60%); PS or
pulmonary valve pulmonary atresia (40%)
[*] [†]
Great arteries Transposition (70%), either D- or L- Normal great arteries (85%);
transposition (15%)
[†]
ECG Normal P axis or in the +90 to +180 degree Superior P axis (70%)
quadrant
study.
Management
• Treatment should be determined by the nature and severity of
the associated cardiac and extracardiac lesions.
• Most cardiac operations for patients with isomerism are
palliative in nature, since normal anatomy is rarely achieved.
• Mortality rates remain high for patients with heterotaxy
syndrome.
• Patients with left isomerism in general have less severe
cardiac malformations than those with right isomerism and,
hence, more chance of biventricular repair.
• For almost all patients with right isomerism, and for many with
left isomerism, biventricular repair will not be feasible, and all
palliative protocols are then staging procedures towards a
Fontan-type repair
FIGURE 6−37. Stages 2 & 3 of HLHS surgical management.
160
Univentricular heart
Definition
The concept of univentricular heart (UVH) moved from hearts
with only one ventricle connected with atria (e.g.double inlet
ventricle) to hearts not amenable to biventricular repair, namely
hearts with two ventricles unable to sustain separately
pulmonary and systemic circulations in sequence. In the latter
definition, even hearts with one hypoplastic ventricle are
considered “functional” univentricular hearts.
Pathophysiology
• The consensus of the STS-Congenital Heart Surgery and the

European study group database proposed that the
nomenclature of UVH include:
Ø double inlet left and right ventricles,
Ø absence of one AV connection,
Ø common AV valves,
In which; Univentricular connection is present when the atria
are connected predominantly with one ventricular chamber
due to absence of one AV valve (absent connection) or to the
presence of two valves or of a common valve predominately
draining in one ventricle (double inlet connection). In both
conditions, one ventricle is of good size and the other is
hypoplastic, missing the inlet portion, and the two ventricular
cavities are separated by an interventricular septum.
The main ventricular chamber may be of right or left
morphology or, in exceptional cases, indeterminate, which
means that the ventricular septum failed to develop.
The description of the side of the absent connection (rightor
left), associated with the identification of the septal position
of the ventricle (d- or l-loop) are to be preferred to the
nomination of the malformation as tricuspid in mitral atresia.
Infact, in hearts with absent right AV connection and d-loop,
the patent left AV valve is a mitral valve, but in case of l-loop,
the patent left AV valve is a tricuspid valve.
And;
Ø Hearts with only one well developed ventricle as unbalanced
AV canal and complex conditions with heterotaxy syndromes.
In which; Both ventricles possess all the morphological
components but where one ventricle is tiny and unable to sustain
the circulation
• This nomenclature does not include hypoplastic left heart
syndrome (HLHS), pulmonary atresia with intact ventricular
septum and certain other biventricular hearts which also
undergo the stages of “univentricular repair”.
• The physiology of UVH depends upon certain key anatomical
factors:
Ø Obstruction to systemic or pulmonary outflows.
Ø Obstruction to ventricular inflow and atrial septum and
abnormal systemic or pulmonary venous return.
Ø Amount of pulmonary blood flow and pulmonary
vascular resistance.
Ø A-V valve regurgitation
With severe systemic outflow obstruction, the patient is
dependent on right-to-left shunting through a patent ductus
161
arteriosus to maintain systemic output. In contrast, with critical

pulmonary outflow obstruction, pulmonary blood flow is
dependent on left-to-right shunting across a patent ductus
arteriosus. Systemic and pulmonary blood flows are
interdependent and determined by resistances of the 2
circulations
With obstruction to pulmonary venous return, severe pulmonary
hypertension may ensue. In the presence of an atretic or
critically stenotic AV valve, unobstructed communication between
both venous inflows and the single ventricle requires an
unrestrictive atrial septal defect (ASD). With an atretic mitral
valve, a restrictive ASD is physiologically similar to pulmonary
venous obstruction. In tricuspid atresia, the physiological effect
of a restrictive ASD is akin to systemic venous obstruction.
Optimal physiology of the univentricular heart requires good
ventricular function without AV valve regurgitation, an
unrestrictive ASD, and well-balanced systemic and pulmonary
blood flow.
Clinical manifestations hinge on the presence or absence of
pulmonary outflow obstruction. Without pulmonary stenosis,
infants with low pulmonary resistance present with signs and
symptoms typical of large left-to-right shunting, ie, congestive
heart failure and failure-to-thrive. Severe pulmonary stenosis or
atresia may result in profound hypoxemia and cyanosis, however
Diagnosis
• Chest X Ray is particularly helpful in assessment of pulmonary
arterial vascularization and configuration of the great arteries
study.
Management
• General objectives of initial surgical palliation are to provide
unobstructed systemic outflow, unobstructed systemic and
pulmonary venous return, and controlled pulmonary blood
flow.
• In patients with severe pulmonary obstruction or atresia, this
is currently accomplished by an aortopulmonary shunt, such
as a modified Blalock-Taussig shunt, or bidirectional
cavopulmonary anastomosis (Glenn shunt).
• Pulmonary circulations solely dependent on aortopulmonary
collaterals pose particular challenges and may require
unifocalization of collaterals as a component of a staged
surgical approach.
• Initial palliation in patients with unrestrictive pulmonary blood
flow may consist of pulmonary artery banding or division with
creation of an aortopulmonary shunt to limit pulmonary blood
flow. Pulmonary banding has been associated with adverse
outcomes after the Fontan procedure, however, and may
result in subaortic obstruction.
• Relief of any pulmonary venous obstruction by either
reconstruction of stenotic anomalous pulmonary venous
connections or a complete atrial septectomy should be
162
performed as needed.
• The most common procedures used in the relief of systemic
outflow tract obstruction are the DKS and Norwood
procedures. In both cases, the main pulmonary artery is used
as the main pathway for the systemic outflow. In cases when
the systemic outflow obstruction is associated with aortic arch
hypoplasia and/or coarctation of the aorta, then Norwood
procedure is the procedure of choice
• A bidirectional Glenn shunt or superior cavopulmonary shunt is
now performed at about 6 months of age. Obstructions or
distortions to the pulmonary arterial tree are corrected during
this intervention. A Fontan procedure is completed sometime
between 18 months and 4 years of age, which thereby
separates pulmonary from systemic circulations
• Choussat et al (1977) delineated selection criteria to define an
ideal candidate for a Fontan procedure. They described the 10
following criteria, which are occasionally and facetiously
referred to as the 10 commandments for an ideal Fontan
operation.
1. Age older than 4 years
2. Sinus rhythm
3. Normal systemic venous return
4. Normal right atrial volume
5. Mean pulmonary artery pressure less than 15 mm Hg
6. Pulmonary arteriolar resistance less than 4 Wood
units/m2
7. Pulmonary artery–aorta ratio more than 0.75
8. Left ventricular ejection fraction more than 0.60
9. Competent mitral valve
10. Absence of pulmonary artery distortion
• Due to limitation in donor availability, cardiac transplantation
cannot provide a comprehensive treatment option for those
patients with single-ventricle physiology, but has become the
bail-out strategy for selected patients with a failed palliative
strategy either with or without preserved ventricular function.
Nevertheless, it should be understood that this does not
constitute a cure but rather a more manageable form of
palliation, which may be associated with a better quality of life
163
FIGURE 6−34. Algorithm for the management of FSV.
164
166
Pulmonary Hypertension
Definition
Normal PAPsystolic ≤30mmHg, mean PAP ≤25 (measured via
cath) -Cor Pulmonale = RVH &/or RV dilation
--increases w elevation bc pulm htn due to pulm parench/vasculature
dz. (Thus, not related to MS or LV failure
Noninvasive Dopplar method often overestimates PAP causing PHTN & RVH…)
-using Bernoulli equation & tricuspid regurg jet
velocity, w RA P assumed to be 10mmHg, the mean PA
systolic P is 28.3 +/-5mmHg , with 95%ile of 37mmHg. Thus Physiology
on Doppler 36-40mmHg is the upper limit for mild PA htn -PVR d/o cross sectional area of small musclar
arts/arterioles; als on degree of stenosis of pulm arts,
Etiology hypoplastic lungs, bld viscosity, total lung mass,
-↑PBF extramural vssl compression.
-CHD w LàR shunt (=hyperkinetic pulm htn) – -Normal PVR = 1 Wood unit (= 1/10 of SVR)
VSD, PDA, EC defect -Exerciseà ↑↑PBF p small ↑PA pressure, w ~=LA P
-alveolar hypoxia -Endothel Cellsà balanced release of NO
-Pulm parench dz (vasodilator) & endothelin (constrictor) to regulate
-pna, hypoplastic lungs (ex p CDH), vascular tone
bronchopulm dsplasia, Interstial lung dz, Wilson-Mikity synd -PGI2, PGE1 dilate, PGF2a & PGA2
-Airway obstruction constrict
-Upper- large tonsils, tongue, small jaw, -Serotonin- constrict & ↑sm muscle
laryngotrach’malacia, OSA hypertrophy; it ↑NO in Nl endothel cells but not in
-Lower- asthma, CF dysfxl endothelial cells
-↓Ventilatory Drive- ↓CNS, obesity hypovent’n synd -Angiotensin II- constrictor, made by ACE
-Resp Muscle dz- kyphoscoliosis, paralyzed skel from Ang I in endothel cells
muscle -hypoxia induced vasoconstriction- à ↓NO,
-High altitutde ↑endothelin, thus pAO2 is major cause of pulm
-↑Pulm vn P arteriolar tone
-MS, cor triatratum, TAPVR w vns return obst, ch L -acidosis- ↑PVR
hrt fail, L side obst lesion- AS, CoAo -alpha R’à constrict, betaR’à dilate. Alpha
-Primary pulm vascular disease R’ blocker thus ↓PVR
-PPHN -hi altitude w low alv O2 tensionà constrict,
-1y pulm htn but this varies a lot.
-Pulm parenchyma/vasculature dz Pathophysiology
-thromboembolism-sickle, thrombophelbitis -RH fails if sudden severe phtn bc it is unprepared
-CT dz- scleroderma, SLE, RA, etc (no RVH) – ex acute ppuer airway obstruction in
-Pulm vssl dz- schistosomiasis, sarcoid, histiocytosis infant, adult w massive PE
X -ch phtnà RVHà Pright >Pleft
-↓CO- bc RV dilation/hypertrophyà ↓Q coronary to
Path L side also w ↓LV fx, & because IV septum pushes
-the thin RV cannot sustain pressure load over 40-50mmHg, left onto LV, & dilated RV à ↓LV complianceà
so acute ↑PVR à RV failure, but if slow ↑ canà RV ↑LV end diast pressure & LA pressureà worse
hypertrophy pulmonary vasoconstriction
-Hyperkinetic Pulmonary Htn- LàR shunt (PDA, VSD)à -sudden ↑PVRà ↓pulm vn return to LAà ↓BP
↑PBF, thus systemic P to PAà compensatory (unless there is a RàL shunt)
vasoconstriction w ↑PVR (w/o ità ↑PBF w CHF), too much -pulm edema bc small arteriole walls prox to the
vasoconstrictors by endothel cells are made. Reversible if u hypoxically constricted arterioles have disrupted
fix the problem. If untreated, à ↑↑pulm htn w cyanosis walls (c/s downstream plugged up, so water forced
when the shunt reverses to RàL bc Pright becomes > Pleft, = out upstream…)
Eisenmenger’s syndrome (pVOD Pulmonary Vascular
Obstructive Disease).
-Alveolar Hypoxia- acute/ch ↓pO2 in alveolar capsà strong
vasoconstriction, which is worsened by the ↓pH,
↑Endothelin, ↓NO
-Pulmonary Vn Htn- (MS, TAPVR, AS, CoAo) ↑P pulm
vnsà reflex constriction of pulm artsà ↑PAP to maintain
pressure gradient across the lung
-Primary Pulm Vsc Dz- similar Sx to Eisenbengers but w/o 166
the cardiac lesion, due to ↓cross sectional area of pulm vsc
167
Hx EKG
-DOE, tired, +/-HA -RAD w RVH, +/-strain if severe, then RAH
-syncope/presyncope, CP w exertion, =advanced dz w a
fixed CO CXR
-CHF/CHD as infant à c/s Eisenmenger’s -Nl/slightly large if RA size, CM only if CHF
-cough/wheezing if underlying lung dz -PA segment & dilated hilar vssls w clear lung fields
-hemoptysis- c/s pulm infarct p thrombosis (late, fatal) -pulm edema if acute exacerbation
PE ECHO
-cyanosis, clubbing, distended neck vn, prominent a wave -big RA, RV
-RV lift or tap @ L parasternal area -thick IV septum, abNl septal motion bc of RV P
-Single S2, narrow split, w loud P2; ejection click & early overload
diast decrescendo murmur (PR) @ mid-LSB. Holosystolic -thick RV free wall, RV dysfx
murmur of TR at LLSB -PA Pressure quantification- …
-R side fail- hepatomeg, ankle edema
-arrhythmias (late) Exercise Stress Testing- 6min walking test.
Diagnosis
-H&P, EKG, CXR, Echo, Cath- is it due to constriction (“responders”) or permanent
changes in pulm arerioles (“nonrsesponders”) after using tolazline (alpha R blocker) &
O2, NO…
-Angiographyà abrupt taper of small arts, reduced background cap filling…
Mgt
-Must prevent & Tx root cause bc u can’t do much for the vascular obstructive dz
àCorrect the CHD, large shunts; T&A for OSA as cause, Tx the CF/asthma/pna/BPD
àprevent further PAP ↑
-avoid strenuous exertion, isometric activities (wtlifting), high altitude/flights
-O2 prn
-avoid vasoconstrictors (degongestants w alpha R ag)
-avoid pregnancyà ↑bld volume & O2 consumption, ↑PE risk, syncope/arrest.
OCP’s also ↑phtn, so surgical contraception is preferred
-CHF- Tx w digoxin (↑RV fx), diuretics (↓Sx), low salt diet
-Cardiac arrhythmia Tx
-Partial erythropheresis- for polycythemia & HA
-flu shot annually
-avoid nitroglycerin for angina bc it will ↑pain
àantigoagulate- warfarin if thromboembolic dz, or ASA
àResponders – Vasodilate
-Nifedipine (CCB) helps 40%kids w 1y PHTN, xx ↓BP
-Prostacyclins- ↑survival & QOL for pt w 1y PHTN, Eisenmengers, CLD;
(Flolan…), use via continuous infusion; xx = flushing, HA, nausea, diarrhea, jaw pain
-Endothelin R antagonist- Bosentan- 1y PHTN, Eisenmengers
-Sildenafil- PDE inhibitor, equally as good as inhaled NO
-inhaled NO- lowers PA in RDS, PPHTN, persistant 1y pulm htn of newborn
àNonresponders- if PVR is fixed, including 1y PHTN
-NO inhaled may help
-Atrial septectomyà ↑survival rate, ↓syncope via a RàL shunt
-Lung transplant
167
List of Books
1. 1 Mostafa, EA; Elnahas, Y; Elmidany, AA; El-Sayed, HH; Kamel, WI; Mansour, SA.
Perspectives in Cardiovascular and Thoracic Surgery
Volume 1: Fundamentals of Cardiac Surgery, 2e, DVD book. 558 pages
Elnasr Publishing Co for Digital Education. 2016
ISBN: 978.977.90.5802.3
2. 2Mostafa, EA; Elnahas, Y; Elmidany, AA; El-Sayed, HH; Kamel, WI; Mansour, SA.
Volume 2: Congenital Heart Surgery, 2e, DVD book. 382 pages
ISBN: 978.977.90.5803.0
Volume 3: Adut Cardiac Surgery,2e, DVD book, 574 pages
ISBN: 978.977.90.5804.7
Volume 4: General Thoracic Surgery, 2e, DVD book, 524 pages
ISBN: 978.977.90.5805.4
Volume 5: Circulatory Support and Transplantation, 1e, DVD book, 144 pages
ISBN: 978.977.90.5806.1
6. 6Mostafa EA
Perspectives in Thoracic and Cardiovascular Surgery
Volume I: General Thoracic Surgery, 1 e.
Stallion Publishing Co., Egypt, 1996.
ISBN: 977-17-1311-6
7. 7Mostafa EA
Volume II: Pediatric Cardiac Surgery, 1e.
Stallion Publishing Co., Cairo, Egypt, 1997.
ISBN: 977-17-1312-4
8. 8Mostafa EA
Volume III: Adult Cardiac Surgery, 1e.
Nasr Publishing Co., Cairo, Egypt, 2005.
ISBN: 977-17-1313-2
9. 9Mostafa, Ezzeldin A; Elnahas, Yasser.
Ain Shams Lecture Notes on Cardiovascular and Thoracic Surgery
Volume 1: Fundamentals of Cardiac Surgery, 2e, 196 pages
ISBN: 978.977.90.5817.7
10. 1 Mostafa, Ezzeldin A; Elmidany, Ashraf A.

0Ain Shams Lecture Notes on Cardiovascular and Thoracic Surgery
Volume 2: Congenital Heart Surgery, 2e, 167 pages
ISBN: 978.977.90.5818.4
11. 1 Mostafa, Ezzeldin A; Mansour, Sherif A.

1 Ain Shams Lecture Notes on Cardiovascular and Thoracic Surgery
Volume 3: Adut Cardiac Surgery,2e, 117 pages
ISBN: 978.977.90.5819.1
12. 1 Mostafa, Ezzeldin A; El-Sayed, Hany H.

Volume 4: General Thoracic Surgery, 2e, 171 pages
ISBN: 978.977.90.5820.7
13. 1 Mostafa, Ezzeldin A; Kamel, Waleed I.

Volume 5: Circulatory Support and Transplantation, 1e, 54 pages
ISBN: 978.977.90.5821.4
14. Mostafa, Ezzeldin A

Spotlights on Cardiovascular & Thoracic Surgery
ISBN: 978.977.90.5807.8
15. Mostafa, Ezzeldin A

Spotlights on Cardiovascular & Thoracic Surgery
16. 1 Mostafa, Ezzeldin A

4Spotlights on Cardiovascular & Thoracic Surgery for House Surgeons

2 AinShamsLectureNotes 2020 CHS

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Part II

Ain Shams Lecture Notes

Ezzeldin A. Mostafa, MD, PhD, MBA

Volume II: Congenital Heart Surgery (Second Edition)

Cover design: Marwa M. Azzam, Cairo, Egypt.

Typesetting by: Ezzeldin A. Mostafa

Digital education design: Ihab M. Abdelmonaem

Printed in Egypt by: Elnasr Publishing Co. for Digital Education

General Abbreviations & Acronyms

2 Acyanotic, without Shunt, Left Side

3 Acyanotic, without Shunt, Right Side

4 Acyanotic, with L®RS

5 Cyanotic with ­ PBF

6 Cyanotic with ¯ PBF

7 Cyanotic with Pulmonary Hypertension

Congenital Heart Surgery Spring 2016

Table of Contents Blue, well-perfused baby .................................. 10

Congenital Heart Surgery Spring 2016

Unit Objectives General Cardiology, Segmental

Congenital Heart Surgery Spring 2016

Definitions & Nomenclatures

Segmental Anatomy & Nomenclature

3 main segments to the heart

Approach to Describing Cardiac in 10 Steps:

Van Praagh Andersonian

1. Thoraco-abdominal situs - describe lungs and abd viscera

Congenital Heart Surgery Spring 2016

4. Atrial Situs Anderson focuses only on the

Situs Solitus, Inversus, or Ambiguous

Congenital Heart Surgery Spring 2016

-MV attaches via 2 distinct pap muscles, to LV free wall

6. AV Alignments and Connections

Congenital Heart Surgery Spring 2016

-Bilateral Conus = in DORV, rarely in TGA

9. Semilunar Valve Relationship

10. Associated Anomalies

Sequential Segmental Analysis

Congenital Heart Surgery Spring 2016

-Pulmonary Sidedness Bilateral right-sidedness and

Congenital Heart Surgery Spring 2016

Morphology of Cardiac Segments

SVC- +/- LSVC

Congenital Heart Surgery Spring 2016

-R Arch has mirror image branching relative to a Nl L arch...

Truncus Arteriosus = Common Truncal Artery

Positions of Cardiac Segments

3. Atrio- ventricular concordance (looping):

Congenital Heart Surgery Spring 2016

ambiguus, the concordance / discordance concept is not applicable.

• Malposition of the Great Arteries (MGA): the great arteries are

• Single (Common) Ventricle: MGA applies accurately, but TGA does

Congenital Heart Surgery Spring 2016

General Pathologic Considerations

Classification of congenital heart disease

Congenital Heart Surgery Spring 2016

Congenital Heart Surgery Spring 2016

Clinical Picture & Diagnostic Considerations

Diagnosing CHD in the Sick Infant

Shock - Not enough systemic flow

Blue, shocky infant

Happy Cyanosis - not enough pulmonary flow

Congenital Heart Surgery Spring 2016

Sick Cyanosis - pulmonary venous congestion

5 Cyanotic with PBF