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Perspectives in Cardiovascular & Thoracic Surgery Volume III: Adult Cardiac


Surgery (ACS)

Book · January 2017

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Perspectives in
Cardiovascular
&
Thoracic
Surgery
Volume III: Adult Cardiac Surgery (Second Edition)
Ezzeldin A. Mostafa, MD, PhD, MBA
Professor of Cardiovascular & Thoracic Surgery
Department of Cardiovascular & Thoracic Surgery
Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Yasser M. Elnahas, MD, PhD
Associate Professor of Cardiovascular & Thoracic Surgery
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.

Hany H. Elsayed, MD, PhD, FRCS(CT)
Associate Professor of Cardiovascular & Thoracic Surgery
Department of Cardiovascular & Thoracic Surgery
Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Waleed I. Kamel, MD, PhD
Lecturer of Cardiovascular & Thoracic Surgery
Department of Cardiovascular & Thoracic Surgery
Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Sherif A. Mansour, MD, PhD, FRCS(CT)
Lecturer of Cardiovascular & Thoracic Surgery
Department of Cardiovascular & Thoracic Surgery
Faculty of Medicine, Ain Shams University, Cairo, Egypt.





Copyright Information
Perspectives in Cardiovascular & Thoracic Surgery

Volume II: Congenital Heart Surgery (Second Edition)

Copyright © 2017 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

NOTICE
Medicine is an ever-changing science. As new research and clinical experience broaden our
knowledge, changes in treatment and drug therapy are required. The authors and the publisher
of this work have checked with sources believed to be reliable in their efforts to provide
information that is complete and generally in accord with the standards accepted at the time of
publication. However, in view of the possibility of human error or changes in medical sciences,
neither the authors nor the publisher nor any other party who has been involved in the
preparation or publication of this work warrants that the information contained herein is in
every respect accurate or complete, and they disclaim all responsibility for any errors or
omissions or for the results obtained from use of the information contained in this work. Readers
are encouraged to confirm the information contained herein with other sources. For example and
in particular, readers are advised to check the product information sheet included in the package
of each drug they plan to administer to be certain that the information contained in this work is
accurate and that changes have not been made in the recommended dose or in the
contraindications for administration. This recommendation is of particular importance in
connection with new or infrequently used drugs.

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



All rights reserved. Usage subject to terms and conditions of license.

Perspectives in Cardiovascular & Thoracic


Surgery
Volume II: Adult Cardiac Surgery

Table of Content


Foreword to the Second Edition by Dr Hamdy M. El-Sayed

Foreword to the First Edition by Dr Hamdy M. El-Sayed

Preface to the Second Edition

Preface to the First Edition

Prologue

List of Symbols, Abbreviations and Acronyms

Chapter 1 Valvular Heart Diseases & Surgery 2

Chapter 2 Ischemic Heart Diseases & Surgery 200

Chapter 3 Aortic Diseases & Aortic Surgery 388

Chapter 4 Pericardial diseases & Surgery 502

Chapter 5 Cardiac Tumors 536























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In contrast, Perspectives in Cardiovascular &
Thoracic Surgery consolidates many of the topics,
Foreword to the Second Edition regardless of their complexity, from definition to
management, in a clear, concise and instructive
Over the years I have had the pleasure of writing way, intermixed with the most recent guidelines.
forewords for the previous edition of the series of Thus, over 200 easily accessible tables dissect and
Perspectives in Cardiovascular & Thoracic Surgery summarize the key points of all the latest
that I considered to be timely and to fulfill guidelines.
important objectives. Without hesitation, I would
say that these series, by Ezzeldin A. Mostafa and his 2) Rapidly evolving scientific knowledge, including
coauthors, is the most outstanding book for which I the value of new diagnostic and management
have had the pleasure to write a foreword. approaches and their incorporation in practicing
guidelines, makes it difficult for the cardiovascular
This is the third CD book of the series about Adult and thoracic surgery specialist to be aware of the
Cardiac Surgery (ACS). Further, this is probably the latest clinical evidence-base.
book that better serves the specialist in day-to-day
practice than any other written in the last two 3) A novelty of this book is the “user-friendly, at
decades. This is not just a textbook; it is an glance” way of presentation that it makes it very
extraordinary “toolkit” in the context of an evidence useful to the practicing cardiovascular and thoracic
based cardiovascular and thoracic surgery practice surgery specialist. Useful because of its combination
in the midst of rapidly evolving scientific knowledge of succinctness and clarity, the book is up to date in
and guidelines. every aspect of the cardiovascular and thoracic
surgery science, and particularly on the most recent
Because of the need to integrate current knowledge recommendations. Thus, these recommendations
on evidence-based cardiovascular and thoracic are summarized in tables derived from the
surgery, about three years ago, under the auspices guideline documents and incorporated in the
of the American Heart Association, they published a appropriate diagnostic or management sections of
book that included the most recent guidelines. I the 7 comprehensive chapters.
believe that such integration was a step forward for
the practicing cardiologist; indeed, in a “synopsis”
fashion, this aspect is well served in Perspectives in This book is a tribute to the skill of Dr Ezzeldin A.
Cardiovascular & Thoracic Surgery. However, in the Mostafa as editor-in-chief and the five co-editors
excellent compendium of my colleagues, three new who also served as the only authors. This limited,
components are incorporated, which we can but unified and hardworking known authorship is,
describe as the “jewel” of the book: a very succinct without doubt, a great part of the success. It is with
definition, classification, pathophysiology, great pleasure that I pen these words to relate my
diagnosis, management, and need of specific clinical enthusiasm for their work as a remarkable addition
investigation of the various disease entities. to the cardiovascular and thoracic surgery field.

These additional three components, about the Adult Hamdy M. El-Sayed, FRCS, FRCP
Cardiac Surgery (ACS), volume #2 of Perspectives in Professor of Cardiovascular &Thoracic Surgery
Cardiovascular and Thoracic Surgery, is so unique, Ain Shams University Hospitals,
deserve a brief description. Faculty of Medicine,
Ain Shams University.
1) In regard to the various disease entities, general
textbooks tend to employ, from definition to
management, a rather long and descriptive format.
Foreword to the First Edition




Preface to the Second Edition When such were not available, they have been
specially drawn. Key concepts and key for
This is the third CD book of the series of diagnosis are highlighted in italics and basic terms
Perspectives in Cardiovascular & Thoracic Surgery are in bold when first mentioned. Bullets and
about the Adult Cardiac Surgery (ACS). It is numbers are used to break down complex processes.
designed to be a concise discussion of the essential
knowledge needed to diagnose and manage We would like to reemphasize again, this is not a
congenital cardiovascular diseases. It cannot be text book, but it is an intermediary educational aid
considered a condensed textbook because detailed for the postgraduate student that affords a rapid
pathophysiologic discussions are omitted; there are and easily digestible format and allows the reader
no chapters on diagnostic techniques; and rare or rapid assimilation of the basic thought process in in
obscure entities are not included. Also, it is not a clinical situations in surgical management of
cardiac therapeutics text because diagnostic congenital heart diseases. The major traditional
techniques, prevention strategies, and prognosis are resources of information, namely journals and
fully discussed. textbooks, remain the cornerstone to serve to
update sources and extend the basic framework of
INTENDED AUDIENCE knowledge
Our aim has been to aid junior staff in their daily
practice with clear and concise information in a Our hope is that the book is found useful and
convenient and accessible format i.e., easy-to- improves patient care. Also, we hope it is an
reference book. It serves as an authoritative state- educational tool that improves knowledge of
of-the art reference for trainees in adult cardiac congenital heart diseases. Finally, we hope it
surgery, and adult cardiologists, anesthetists, stimulates clinical research in areas where our
intensivists, perfusionists, pathologists, imaging knowledge is incomplete.
experts, nurses, technicians and others.
We also wish to acknowledge our spouses who
COVERAGE inspired and supported us in our educational
The format of this book, “Perspectives in pursuits; our partners, trainees, and colleagues who
Cardiovascular & Thoracic Surgery”, follows that of carry the field forward; and our patients who put
the previous edition. In each disease, the their trust and hope in our hands. A special thank to
overwhelming emphasis remains on surgery, our teacher and mentor Dr. Hamdy M. El-Sayed,
although it includes definition, general pathologic cardiothoracic surgery pioneer and innovator who
considerations, clinical picture and diagnostic has written, for the second time, a superb foreword
modalities referable to the particular disease being to this second edition.
discussed. Several design features are included to
make the text easy to use. High-yield clinical pearls Finally, we dedicate this volume to our professors of
are integrated throughout the text; clinical cardiothoracic surgery who had the wisdom and
examples highlight the relevance and application of vision to induce the adult heart surgery unit at Ain
surgical concepts. We hold a strong conviction Shams University Hospitals.
concerning the importance of illustrations,
algorithms and flow charts. Approximately 450 Ezzeldin A. Mostafa
figures illustrate essential processes; explanatory Yasser M. Elnahas
figure legends allow figures to be used for review. Ashraf A. Elmidany
Experience has emphasized repeatedly that Hany H. Elsayed
students both appreciate and utilize them Waleed I. Kamel
appropriately, and in each instance the figures have Sherif A. Mansour
been selected from the very best in the literature.
Preface to the First Edition


incredibly complex and intricate concepts yet at
times unable to recognize the obvious and simple.
Prologue Therefore, try to use the KISS principle. Keep it
Simple and Stupid. With this goal planner, the
The entire field of cardiovascular and thoracic blueprint of success, you can unlock your true
surgery has witnessed an era of rapid scientific potential to awaken the true power of your brain.
progress, accompanied by continuous technological
and applied innovation. This occurs against a The KISS or the simpleology is the express track or
backdrop of increasing emphasis on the importance goal planner to improve memory, reading all these
of evidence-based practice, and rapid development topics, and super intelligence.
of guidelines by major professional societies. The
resultant expansion of our body of knowledge by An idiom and a mnemonic should be recalled for
evidence-based recommendations interjects a new how to use the book. As regards the idiom “ A
set of challenges for the practicing clinician with picture is worth one thousand words”.
ever-extensive clinical responsibilities.
As for the mnemonic: Nobody Can Give Money To
In order to practice evidence-based medicine, ATM (Automatic Teller Machine); where Nobody:
information must be easily accessible and, more Names, Numbers and Mnemonic (silent m); Can:
importantly, easily retrievable when the need Charts; Give: Guidelines; Money: Management; To:
arises; this may not always be easy with the current Tables; and ATM: Algorithms, Tables, and
pace of dissemination of knowledge. The rationale Management protocols (MIS); Medical,
for writing this book reflects exactly this need, both Interventional, and Surgical. Additional materials
ours and that of our potential readers: to organize (Ain Shams lecture wikinotes, wikislides and CTS
our continually evolving knowledge on often test bank) are available on the website: www.
diverse cardiovascular and thoracic issues, in our ezzeldinmostafa.com.
environment of networked and facilitated
communication.
HOW TO USE THE BOOK?
The prerequisites of informed clinical practice are: This is the second CD book of the series of
a satisfactory background of basic knowledge of Perspectives in Cardiovascular & Thoracic Surgery
disease entities, remaining up-to-date on important about the fundamentals of cardiovascular and
clinical trials and emerging scientific evidence that thoracic surgery (CTS) contains four parts for each
shape current diagnosis and therapy, and 7 chapters.
acquaintance with current practice guidelines from 1. Lecture Notes: can be downloaded from the
established professional societies such as the website: www. ezzeldinmostafa.com.
Society of Thoracic Surgery/American Association 2. Book Chapter: in details; the text on the left-
of Thoracic Surgery (STS/AATS) and the European side of the screen and the diagram on the right-side
Association of Cardiothoracic Surgery (EACTS), of the screen.
among many others. 3. Wikislides, powerpoint presentation for the
topics in pdf files.
In other words, to provide a clinical tool that can be 4. O & A (Questions & answers) on the chapter ‘s
used in everyday clinical practice as a concise guide topics.
to what we know and, more importantly, what we
do not know, and what we think we know. To quote Ezzeldin A. Mostafa
Mark Twain, “what gets us into trouble is not what
we don’t know, it is what we know for sure that just
ain’t so.”

How to unleash your unlimited potential and
develop a super memory? An amazing thing, the
human brain, is capable of understanding

ACAS Asymptomatic Carotid Atherosclerosis Study
Symbols, Abbreviations and ACC American College of Cardiology
Acronyms ACCF American College of Cardiology Foundation
ACCF American College of Cardiology Foundation
ACCOMPLISH Avoiding Cardiovascular
Symbols Events Through Combination Therapy in Patients
Living With Systolic Hypertension
≈ approximately ACE angiotensin converting enzyme
§ cross-reference ACE angiotensin-converting enzyme
° degrees ACE angiotensin-converting enzyme
® Trade name AChR acetyl cholinesterase receptor
+ ve positive ACIP Asymptomatic Cardiac Ischaemia Pilot
± with/without ACS acute coronary syndrome
< less than ACS acute coronary syndrome
> more than ACST Asymptomatic Carotid Surgery Trial
− ve negative ADA adenosine deaminase
≤equal or less than ADA American Diabetes Association
≥ equal or more than ADH antidiuretic hormone
DDx or DD differential diagnosis ADP adenosine diphosphate
ê decreased ADP adenosine diphosphate
Dx Diagnosis AF atrial fibrillation or AFib
Hx History AHA American Heart Association
é increased ALI acute limb ischaemia
è leads to or results in AMI acute myocardial infarction
M:F Male to Female ratio AMI acute myocardial infarction
ò Female ANA anti-nuclear antibody
ô Male ANS autonomic nervous system
Tx Treatment AP anteroposterior
α alpha APC antigen presenting cell
β beta APTT activated partial thromboplastin time
δ delta ARB angiotensin II receptor antagonist
Δ triangle ARDS acute respiratory distress syndrome
µ mean ARF acute respiratory failure
ARI acute renal insufficiency
ART Arterial Revascularization Trial
ASCOT Anglo-Scandinavian Cardiac
Abbreviations and Acronyms Outcomes Trial
This is the list of abbreviations and acronyms which
ASSERT Asymptomatic atrial fibrillation and
occur frequently in the text. Those less commonly
Stroke Evaluation in pacemaker patients and the
used are explained where they occur.
atrial fibrillation Reduction atrial pacing Trial
ASTRAL Angioplasty and Stenting for Renal
Artery Lesions trial
ATP adenosine triphosphate
AV atrioventricular
b.p.m. beats per minute
BARI 2D Bypass Angioplasty Revascularization
201
TI thallium 201
Investigation 2 Diabetes
2D two-dimensional
BASIL Bypass versus Angioplasty in Severe
3D three-dimensional
99m Ischaemia of the Leg
Tc technetium-99m
BEAUTIFUL Morbidity-Mortality Evaluation of the
ABCB1 ATP-binding cassette sub-family B
If Inhibitor Ivabradine in Patients With Coronary
member 1
Artery Disease and Left Ventricular Dysfunction
ABG arterial blood gas
bFGF basic fibroblast growth factor
ABI ankle-brachial index
bid twice a day (bis in die)
ABI ankle–brachial index


BIMA bilateral internal mammary artery CLL chronic lymphocytic leukemia
BiPAP biphasic positive pressure ventilation cm centimeter/s
BMI body mass index CMR cardiac magnetic resonance
BMS bare metal stent CMR cardiac magnetic resonance
BNP B-type natriuretic peptide CMR cardiac magnetic resonance
BOA Dutch Bypass Oral Anticoagulants or Aspirin CMV cytomegalovirus
BP blood pressure CMV cytomegalovirus or controlled mechanical
BP blood pressure ventilation
BSA bovine serum albumin CN cyanide
BUN blood urea nitrogen CNS central nervous system
C/O complaint of CO cardiac output
Ca2+ calcium COPD chronic obstructive pulmonary disease
CABG coronary artery bypass graft CORAL Cardiovascular Outcomes in
CABG coronary artery bypass grafting Renal Atherosclerotic Lesions
CAD coronary artery disease CORONARY The CABG Off or On Pump
CAD coronary artery disease Revascularization Study
CAPRIE Clopidogrel versus Aspirin in Patients COURAGE Clinical Outcomes Utilization
at Risk for Ischaemic Events Revascularization and Aggressive Drug Evaluation
CAPRIE Clopidogrel vs. Aspirin in Patients at COURAGE Clinical Outcomes Utilizing
Risk of Ischaemic Events Revascularization and Aggressive Drug Evaluation
CAPTURE Carotid ACCULINK/ACCUNET Post COX-1 cyclooxygenase-1
Approval Trial to Uncover Rare Events COX-2 cyclooxygenase-2
CARP Coronary Artery Revascularization CP Child–Pugh
Prophylaxis CPAP continuous positive airway pressure
CAS carotid artery stenting CPB cardiopulmonary bypass
CASPAR Clopidogrel and Acetylsalicylic Acid in CPG Committee for Practice Guidelines
Bypass Surgery for Peripheral Arterial Disease CPG Committee for Practice Guidelines
CASS Coronary Artery Surgery Study CPR cardiopulmonary resuscitation
CASS Coronary Artery Surgery Study CREST Carotid Revascularization
CAVATAS CArotid and Vertebral Artery Endarterectomy vs. Stenting Trial
Transluminal Angioplasty Study CRI chronic renal insufficiency
CAVH continuous arteriovenous hemofiltration CRP C-reactive protein
CBC complete blood count CRP C-reactive protein
CCB calcium channel blocker CSF cerebrospinal fluid
CCS Canadian Cardiovascular Society CT computed tomography
CDC Centers for Disease Control and Prevention CT computed tomography
CEA carotid endarterectomy CT computed tomography
CFR coronary flow reserve CT computed tomography
CHARISMA Clopidogrel for High Atherothrombotic CTA computed tomography angiography
Risk and Ischaemic Stabilization, Management and CTA computed tomography angiography
Avoidance CTA computed tomography angiogram
CHARISMA Clopidogrel for High Atherothrombotic CV cardiovascular
Risk and Ischaemic Stabilization, Management and CVA cerebrovascular accident
Avoidance CVD cardiovascular disease
CHF congestive heart failure CVD cardiovascular disease
CI confidence interval CVP continuous venous pressure
CI confidence interval CVVH continuous venovenous hemofiltration
CK creatine kinase CXR chest X-ray
CKD chronic kidney disease CXR chest X-ray
CKD-EPI Chronic Kidney Disease Epidemiology CYP2C19*2 cytochrome P450 2C19
Collaboration CYP3A cytochrome P3A
CLEVER Claudication: Exercise Versus CYP3A4 cytochrome P450 3A4
Endoluminal Revascularization CYP450 cytochrome P450
CLI critical limb ischaemia


DANAMI Danish trial in Acute Myocardial ET endotracheal
Infarction EtOH ethanol (alcohol)
DAPT dual antiplatelet therapy EUROSCORE European System for Cardiac
DBP diastolic blood pressure Operative Risk Evaluation
DECOPI Desobstruction Coronaire en Post- EUS endoscopic ultrasound
Infarctus EVA-3S Endarterectomy Versus Angioplasty in
DECREASE-V Dutch Echocardiographic Cardiac Patients with Symptomatic Severe Carotid Stenosis
Risk Evaluation EVLW extravascular lung water
DES drug-eluting stents EXACT Emboshield and Xact Post
DHP dihydropyridine Approval Carotid Stent Trial
DIC disseminated intravascular coagulation EXCEL Evaluation of XIENCE PRIME or
DLCO diffusing capacity of the lung for carbon XIENCE V vs. Coronary Artery Bypass Surgery for
monoxide Effectiveness of Left Main Revascularization
DMSO dimethyl sulfoxide solution FAME Fractional Flow Reserve vs. Angiography for
DO2 oxygen delivery Multivessel Evaluation
DRASTIC Dutch Renal Artery Stenosis FB foreign body or feedback
Intervention Cooperative Study FBC full blood count
DSA digital subtraction angiography FDA Food & Drug Administration (USA)
DSE dobutamine stress echocardiography FDG fluorodeoxyglucose
DSWI deep sternal wound infection Fe iron
DUS duplex ultrasound/duplex ultrasonography FEV1 forced expiratory volume in 1 second
DVT deep vein thrombosis FFP fresh frozen plasma
E/M electron microscopy FFR fractional flow reserve
EACTS European Association for Cardio- FFR fractional flow reserve
Thoracic Surgery FiO2 fraction of inspired oxygen
EACTS European Association for FMF familial Mediterranean fever
Cardiothoracic Surgery FREEDOM Design of the Future
EAS European Atherosclerosis Society Revascularization Evaluation in patients with
EASD European Association for the Study of Diabetes mellitus: Optimal management of
Diabetes Multivessel disease
EBV Epstein–Barr virus FVC forced vital capacity g gram/s
EBV Epstein–Barr virus GALA General Anaesthesia versus Local
ECF extracellular fluid Anaesthesia for Carotid Surgery
ECG electrocardiogram GFR glomerular filtration rate
ECG electrocardiogram GFR glomerular filtration rate
ECHO echocardiogram GFR glomerular filtration rate
ECMO extracorporeal membrane oxygenation GI gastrointestinal
ECST European Carotid Surgery Trial GM-CSF granulocyte-macrophage colony-
ED erectile dysfunction stimulating factor
EECP enhanced external counterpulsation GRACE Global Registry of Acute
EEG electroencephalogram Coronary Events
EF ejection fraction HACEK Hemophilus, Actinobacillus,
EKG electrocardiogram Cardiobacterium, Eikenella, Kingella
EMA European Medicines Agency HAR hyperacute rejection
EMG electromyogram HbA1c glycated haemoglobin
EPD embolic protection device HbA1c glycated haemoglobin
EPO erythropoietin HCV hepatitis C virus
ERT emergency room thoracotomy HDL high density lipoprotein
ESC European Society of Cardiology HDL high-density lipoprotein
ESC European Society of Cardiology HDL-C high density lipoprotein cholesterol
ESH European Society of Hypertension HHV human herpesvirus
ESR erythrocyte sedimentation rate HIT heparin-induced thrombocytopenia
ESRD end-stage renal disease HITT heparin-induced thrombocytopenia and
ESRD end-stage renal disease thrombosis


HIV human immunodeficiency virus LDL low-density lipoprotein
HLA human leukocyte antigen LDL-C low density lipoprotein cholesterol
HOPE Heart Outcomes Prevention Evaluation LEAD lower extremity artery disease
HR hazard ratio LFT liver function test
HR hazard ratio LIMA Left internal mammary artery
HR hazard ratio LM left main
HR heart rate LMS left main stem
hr hour LMWH low molecular weight heparin
HRT hormone replacement therapy LPA left pulmonary artery
hs-CRP high-sensitivity C-reactive protein LSV long saphenous vein
HTLV human T-lymphotrophic virus LSVC left superior vena cava
HU Hounsfield units LV left ventricle/ventricular
IABP intra-aortic balloon pump LVEF left ventricular ejection fraction
IC intermittent claudication LVH left ventricular hypertrophy
ICA invasive coronary angiography M/E microscopic examination
ICD implantable cardioverter-defibrillator or MACCEs major adverse cardiac and
International Classification of Diseases cerebrovascular events
ICSS International Carotid Stenting Study MACE major adverse cardiac events
ICU intensive care unit MAO monoamine oxidase
IL interleukin MAP mean arterial pressure
IM intramuscular MASS Medical, Angioplasty, or Surgery Study
IMA internal mammary artery MCV mean corpuscular volume
IMT intima–media thickness MDCT multidetector computed tomography
INPV intermittent negative pressure ventilation MDRD Modification of Diet in Renal Disease
INR international normalized ratio MDT multidisciplinary team
Int intermediate MERLIN Metabolic Efficiency with Ranolazine
IONA Impact Of Nicorandil in Angina for Less Ischaemia in Non-ST-Elevation Acute
IPPV intermittent positive pressure ventilation Coronary Syndromes
ISCHEMIA International Study of Comparative MERLIN-TIMI 36 Metabolic Efficiency with
Health Effectiveness with Medical and Invasive Ranolazine for Less Ischemia in Non-ST-Elevation
Approaches Acute Coronary Syndromes: Thrombolysis In
ITA internal thoracic artery Myocardial Infarction
ITT intention to treat MET metabolic equivalents
IV intravenous mg milligram/s
IVC inferior vena cava MI myocardial infarction
IVIG intravenous immunoglobulins MICRO-HOPE Microalbuminuria, cardiovascular and
IVUS intravascular ultrasound renal sub-study of the Heart Outcomes Prevention
JSAP Japanese Stable Angina Pectoris Evaluation study
JVP jugular venous pressure min minute/s m
K potassium MMF mycophenolate mofetil
KATP ATP-sensitive potassium channels mmol millimole/s
kg kilogram/s L liter/s MMV mandatory minute ventilation
L milliliter/s m MONICA Monitoring of Trends and
L. left-sided Determinants in Cardiovascular Disease
LA left atrium/atrial mPAP mean pulmonary artery pressure
LAD left anterior descending MPI myocardial perfusion imaging
LAO left anterior oblique MRA magnetic resonance angiography
LAP left atrial pressure MRI magnetic resonance imaging
LAP mean left atrial pressure MRSA meticillin-resistant Staphylococcus aureus
LASER is an acronym for Light Amplification by ms millisecond/s
Stimulated Emission Radiation. MVO2 mixed venous oxygen saturation
LBBB left bundle branch block MVV maximal voluntary ventilation
LCE late contrast-enhanced N/E naked eye appearance
LDL low density lipoprotein Na sodium


NASCET North American Symptomatic Carotid po by mouth (per os)
Endarterectomy Trial POBA plain old balloon angioplasty
NBM nil by mouth PPI proton pump inhibitor
NG nasogastric ppo predicted postoperative
NIBP non-invasive blood pressure PPS post-pericardiotomy syndrome
NIPPV non-invasive intermittent positive pressure PRECOMBAT Premier of Randomized Comparison
ventilation of Bypass Surgery vs. Angioplasty Using Sirolimus-
NO nitric oxide Eluting Stent in Patients with Left Main Coronary
nocte at night Artery Disease
NPO withold food and drink by mouth (nil per os) prn pro re nata (as required)
NSAIDs non-steroidal anti-inflammatory drugs PRO-CAS Predictors of Death and Stroke in
NSTE-ACS non-ST-elevation acute coronary CAS
syndrome PS pulmonary stenosis or pressure support
NYHA New York Heart Association PSV pressure support ventilation
O/E on examination PT prothrombin time
OAT Occluded Artery Trial PTA percutaneous transluminal angioplasty
OCP oral contraceptive pill PTCA percutaneous transluminal coronary
OCT optical coherence tomography angioplasty
od once a day (omne in die) PTE pulmonary thromboembolism
OMT optimal medical therapy PTP pre-test probability
ONTARGET Ongoing Telmisartan Alone and in PUFA polyunsaturated fatty acid
Combination with Ramipril Global Endpoint Trial PVC polyvinyl chloride
OR odds ratio PVD peripheral vascular disease
OR operating room or odds ratio PVR peripheral vascular resistance
pa per annum PVRI pulmonary vascular resistance index
PA pulmonary artery or posteroanterior or qid four times a day (quater in die)
physician assistant QoL quality of life
PAD peripheral artery diseases R. right-sided
PAH pulmonary arterial hypertension RA right atrium/atrial
PAH pulmonary arterial hypertension RAAS renin–angiotensin–aldosterone system
PAN polyarteritis nodosa RADAR Randomized, Multicentre, Prospective
PAP pulmonary artery pressure Study Comparing Best Medical Treatment Versus
PAR-1 protease activated receptor type 1 Best Medical Treatment Plus Renal Artery Stenting
PARTNERS Peripheral Arterial Disease in Patients With Hamodynamically Relevant
Awareness, Risk, and Treatment: New Resources Atherosclerotic Renal Artery Stenosis
for Survival RAO right anterior oblique
PAWP pulmonary artery wedge pressure RAP right atrial pressure
PCI percutaneous coronary intervention RAS renal artery stenosis
PCI percutaneous coronary intervention RBB right bundle branch
PCIS post-cardiac injury syndromes RBBB right bundle branch block
PCR polymerase chain reaction RBC red blood cell
PDA posterior descending artery or patent ductus RCT randomized controlled trial
arteriosus REACH Reduction of Atherothrombosis for
PDE5 phosphodiesterase type 5 Continued Health
PDGF platelet-derived growth factor REACH Reduction of Atherothrombosis for
PEA pulseless electrical activity Continued Health
PEEP positive end-expiratory pressure RITA-2 Second Randomized Intervention Treatment
PES paclitaxel-eluting stents of Angina
PET positron emission tomography ROOBY Veterans Affairs Randomized On/Off
PFO patent foramen ovale Bypass
PGE1 prostaglandin E1 RPA right pulmonary artery
PICU pediatric intensive care unit RR risk ratio
Plts platelets RSPV right superior pulmonary vein
PNS peripheral nervous system RSV respiratory syncytial virus



RV right ventricle/ventricular SYNTAX SYNergy between percutaneous
S. & S. symptoms and signs coronary intervention with TAXus and cardiac
s/l sublingual surgery
SA sinoatrial TASC TransAtlantic Inter-Society Consensus
SAM systolic anterior motion TB tuberculosis
SaO2 oxygen saturation of arterial blood TC total cholesterol
SAPPHIRE Stenting and Angioplasty with TEE transesophageal echocardiography
Protection in Patients at High Risk for TEG thrombelastography
Endarterectomy TENS transcutaneous electrical neural stimulation
SAPT single antiplatelet therapy TERISA Type 2 Diabetes Evaluation of
SBP systolic blood pressure Ranolazine in Subjects With Chronic Stable Angina
SBT spontaneous breathing trials TIA transient ischemic attack
SC subcutaneous tid three times a day (ter in die)
SCAD stable coronary artery disease TIME Trial of Invasive vs. Medical therapy
SCAI Society for Cardiovascular Angiography and TIMI Thrombolysis In Myocardial Infarction
Interventions TIVA total intravenous anesthesia
SCBU special care baby unit TLC total lung capacity
SCORE Systematic Coronary Risk Evaluation TMR transmyocardial laser revascularization
SCS spinal cord stimulation TNF tumour necrosis factor
SES sirolimus-eluting stents TOAT The Open Artery Trial
SIMA single internal mammary artery TOS thoracic outlet syndrome
SIMV synchronized intermittent mandatory TRAM transverse rectus abdominis myocutaneous
ventilation TRAPS tumour necrosis factor receptor-
SIR Society of Interventional Radiology associated periodic syndrome
SIRS systemic inflammatory response syndrome TSH thyroid stimulating hormone
SLE systemic lupus erythematosus TTE transthoracic echocardiography
SNP sodium nitroprusside Tx treatment
SPACE Stent-Protected Angioplasty versus U unit/s
Carotid Endarterectomy U/S ultrasound
SPARCL Stroke Prevention by Aggressive U&E urea and electrolytes
Reduction in Cholesterol Levels Study UEAD upper extremity artery disease
SPECT single photon emission computed uIFN-γ unstimulated interferon-gamma
tomography URTI upper respiratory tract infection
spp. species VA vertebral artery
SR survival rate VATS video-assisted thoracic surgery
SSEP somatosensory evoked potential VAVD vacuum-assisted venous drainage
SSFP steady-state free-precession VC vital capacity
SSYLVIA Stenting of Symptomatic VEGF vascular endothelial growth factor
Atherosclerotic Lesions in the Vertebral or VF ventricular fibrillation
Intracranial Arteries vit vitamin
STAR Stent Placement in Patients With VQ ventilation-perfusion
Atherosclerotic Renal Artery Stenosis and Impaired VSD ventricular septal defect
Renal Function VT ventricular tachycardia, VTach
STICH Surgical Treatment for Ischaemic WCC white cell count
Heart Failure WL window level
STIR short-tau inversion-recovery WOEST What is the Optimal antiplatElet and
SVC superior vena cava anticoagulant therapy in patients with oral
SVMB Society for Vascular Medicine and Biology anticoagulation and coronary StenTing
SvO2 mixed venous oxygen saturation WW window width
XM cross match
SVR systemic vascular resistance
yr(s) year (s)
SWI superficial wound infection
SWISSI II Swiss Interventional Study on Silent
Ischaemia Type II


2 1.Valvular Heart Diseases (VHD)

1 Valvular Heart Diseases (VHD)

Rheumatic Heart Diseases (RHD)


countries because of the effects of declining
Chronic rheumatic heart disease (rheumatic economic standards, malnutrition, overcrowding,
valvulitis, inactive) RHD, although rapidly and urbanization.
disappearing in the industrial nation-states, is still It results from single or repeated attacks of
rampant in our country and most of the developing rheumatic fever (RF) that produce rigidity and
countries, which make up more than two thirds of deformity of the cusps, fusion of the commissures,
the world population. It was and still is the or shortening and fusion of the chordae tendineae.
commonst underlying cause of cardiovascular Stenosis or insufficiency results, and both often
disability and death in our country and most of the coexist, although one or the other predominates.
developing countries. RHD is estimated to be The mitral valve alone is affected in 50-60% of
resposible for 30-60% of all cardiac patients cases; combined lesions of the aortic and mitral
admitted to the hospital. The prevelance among valves occur in 20%; pure aortic lesions in 10%.
school children ranges from 1.0 to 10 per 1000, Tricuspid involvement occurs only in association
while the incidence ranges from 10 to 100 per with mitral or aortic disease in about 10% of cases.
100,000, with a high rate of recurrent attacks and The pulmonary valve is rarely affected.
severity. It constitutes the leading cause of death Mnemonic: The relative incidence of valvular
(0.3-8.0 per 100,100 population) from heart involvement in rheumatic heart disease is proportional
disease in the 5-to-24-year-old age group in most approximately to the pressure against which the valve
parts of the world. With this figure, It has been closes, i.e. mitral 100; aortic 70; tricuspid 25; pulmonary
10; or 10:7:2:1.
assumed an estimate of 12 million people affected
Thus, the suggestion is that trauma, as well as
by RF/RHD with 400,0000 deaths annually and
immune factors, determines the local evolution`of
hundreds of thousands disabled young adults.
the process. A history of RF is obtainable in only
Rather than showing evidence of decline, its
60% of patients with rheumatic heart disease.
incidence may even be increasing in a severe,
aggressive and malignant way in some of these

Rheumatic fever (RF)

Rheumatic fever (RF) is an acute, subacute, or more common in males than in females, but chorea
chronic systemic auto-immune connective tissue is seen more frequently in females. RHD is
disease due to some type of hyperimmune reaction estimated to be resposible for 30-60% of all cardiac
either to bacterial allergy or to autoimmunity. That patients admitted to the hospital. The prevelance
is a sequela to beta-hemolytic streptococcal among school children ranges from 1.0 to 10 per
infection as proven by clinical, epidimiologic, 1000, while the incidence ranges from 10 to 100
immunological and prophylactic evidences. For per 100,000, with a high rate of recurrent attacks
unknown reasons it may either be self-limiting, or and severity.
lead to slowly progressive valvular deformity. Predisposing Factors: RF is initiated by an
Q. Why is RF considered as a connective tissue infection with group A hemolytic streptococci,
disease? appearing usually 1-4 weeks after tonsillitis,
Because its anatomic hallmark is damage to nasopharyngitis, or otitis.
collagen fibrils and the ground substances of Macroscopic Appearance: The acute phase of
connective tissue (especially in the heart) RF may involve the endocardium, myocardium,
pericardium, synovial joint linings, lungs, or pleura.
General Pathologic Considerations The mitral valve is attacked in 75-80% of cases,
Incidence: RF is the most common precursor of the aortic valve in 30%, the tricuspid and
heart disease in people under age 50 years. The pulmonary valves in less than 5%. Small pink
peak incidence occurs between ages 5 and 15; granules appear on the surface of the edematous
rheumatic fever is rare before age 4 and after 50. valve. Healing may be complete, or a progressive
In overall incidence, it ranks first (in our country) scarring due to. subacute or chronic inflammation
and third (in western) behind hypertension and may develop over months and years.
atherosclerotic coronary disease. It is somewhat
1.Valvular Heart Diseases (VHD) 3

FIGURE 1−1. Pathogenesis pathway for ARF & RHD. Lancet 2005;366:155-68

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4 1.Valvular Heart Diseases (VHD)

Microscopic Examination: The characteristic lesion is a perivascular granulomatous reaction and


vasculitis
Complications
• Cardiac: CHF (in severe cases), cardiac In the absence of any of the above definite signs,
arrhythmias, pericarditis with large the diagnosis of carditis depends upon the
effusion, cardiac invalidism, and early following less specific abnormalities considered in
or late development of permanent heart relation to the total clinical picture.
valve deformity. 1. ECG changes: P-R prolongation greater
• Pulmonary: rheumatic pneumonitis, than 0.04 second above the patient's
pulmonary embolism and infarction. normal is the most significant abnormality;
RF constitutes the leading cause of death (0.3-8.0 changing contour of P waves or inversion of
per 100,100 population) from heart disease in the T waves is less specific.
5-to-24-year-old age group in most parts of the 2. Changing quality of heart sounds.
world. With this figure, It has been assumed an 3. Pansystolic apical murmur that persists or
estimate of 12 million people affected by RF/RHD becomes louder during the course of the
with 400,0000 deaths annually and hundreds of disease and is transmitted into the axilla.
thousands disabled young adults The Carey Coombs short middiastolic
murmur should be carefully sought.
Clinical Findings & Diagnosis 4. Gallop rhythm: Difficult to differentiate from
the physiologic third sound in children and
A. Major Criteria:
Mnemonic for rheumatic fever is Jones Cafe Pal adolescents -
Carditis-The presence of carditis establishes the 5. Sinus tachycardia out of proportion to the
diagnosis of RF whenever there is (1) a definite degree of fever, persisting during sleep and
history of RF, or (2) valvular disease clearly of markedly increased by slight activity.
rheumatic origin, or (3) a streptococcal infection of 6. Arrhythmias, shifting pacemaker, ectopic
the upper respiratory tract known to have occurred beats.
within the preceding 4 weeks. Carditis is most apt The 2 following signs occur most often in
to be evident in children and adolescents; in adults, association with severe carditis and so are of little
it is often best detected by serial ECG study. Any value in initial diagnosis; occasionally, however,
of the following establishes the presence of they appear before carditis is evident and
carditis. constitute strong presumptive evidence of RF.
1. Pericarditis-Either fibrinous with a pleuritic type a. Erythema marginatum (annulare)-
of precordial, epigastric, or left shoulder pain; Frequently associated with skin nodules. The
friction rub; characteristic ST-T changes on the lesions begin as rapidly enlarging macules that
ECG or with effusion of any degree. It is assume the shape of rings or crescents with clear
uncommon in adults and is at times diagnosed centers. They may be slightly raised and confluent.
by the progressive increase in "heart shadow " The rash may be transient or may persist for long
on serial chest x-rays or by echocardiography. periods.
2. Cardiac enlargement, detected by physical b. Subcutaneous nodules-These are
signs or x-ray, indicating dilatation of a uncommon except in children. The nodules may
weaken inflamed myocardium. Serial x-rays be few or many; are usually small (2 cm or less in
are often needed to detect the change in diameter), firm, and nontender; and are attached to
size. fascia or tendon sheaths over bony prominences
3. Frank CHF, right- and left-sided-right heart such as the elbows, the dorsal surfaces of the
failure is more prominent in children, and hands, the malleoli, the vertebral spines, and the
painful engorgement of the liver is a occiput. They persist for days or weeks, are
valuable sign. usually recurrent, and are clinically
4. Mitral or aortic diastolic murmurs, indicative indistinguishable from the nodules of rheumatoid
of dilatation of a valve ring or the arthritis.
myocardium with or without associated
valvulitis.

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1.Valvular Heart Diseases (VHD) 5

FIGURE 1−2. Acute rheumatic fever (ARF). Pathogenesis & clinical findings. 2017 - The Calgary Guide to
Understanding Disease. www.calgaryguide.com

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6 1.Valvular Heart Diseases (VHD)

Chorea (Sydenham's chorea, chorea minor, and rheumatic manifestations such as erythema
St. Vitus’ chorea) may appear suddenly as an marginatum or chorea.
isolated entity with no "minor criteria" or may
develop in the course of overt RF. Eventually 50% B. Minor Criteria: The following nonspecific
of cases have other signs of RF. Girls are more manifestations of RF are of diagnostic help only
frequently affected, and occurrence in adults is when associated with more specific features.
rare. Chorea consists of continual, nonrepetitive, Fever is always present with arthritis and carditis.
purposeless jerky movements of the limbs, trunk, In subacute or chronic phases it is low-grade and
and facial muscles. Milder forms masquerade as may be continuous or intermittent. Fever is
undue restlessness as the patient attempts to important only as evidence of an inflammatory
convert uncontrolled movements into seemingly process. Certain children and even adults may
purposeful ones. Facial grimaces of infinite variety have normal peak temperatures of 37.5-37.8 C
are common. These movements are made worse (99.5-100 F), and this should not be construed
by emotional tension and disappear entirely during erroneously as "fever”.
sleep. The episode lasts several weeks, Malaise, asthenia, weight loss, and anorexia
occasionally months. may be the only overt effects of a smoldering
rheumatic state but are also characteristic of any
Arthritis-The arthritis of RF is characteristically a chronic active disease.
migratory polyarthritis of gradual or sudden onset Abdominal pain is common. It is variable in site
that involves the large joints sequentially, one and severity and occasionally leads to an
becoming hot, red, swollen, and tender as the unnecessary laparotomy. It may result from liver
inflammation in the previously involved joint engorgement, sterile rheumatic peritonitis, or
subsides. The body temperature rises rheumatic arteritis or may be referred from the
progressively as each successive joint becomes pleura or pericardium.
inflamed. In adults, only a single or a small joint Recurrent epistaxis is believed by some clinicians
may be affected. The acute arthritis lasts 1-5 to indicate "subclinical" RF.
weeks and subsides without residual deformity "Growing pains" in joints, periarticular tis'sues, or
except for the rare persistent arthritis known as muscle insertions may be a symptom of RF
Jaccoud's arthritis. ("arthralgia").
C.Laboratory Findings: These are helpful in
Note: Joint involvement is considered a major 3 ways.
criterion only when definite effusion and signs 1. As nonspecific evidence of inflammatory
of inflammation are present. This is in contrast disease-Sedimentation rate (ESR) and white
to arthralgia, in which pain or stiffness is cell count (WBC or TLC) are almost always
present without these objective signs. Prompt increased during active rheumatic fever except
response of arthritis to therapeutic doses of when chorea is the only clinical sign. Variable
salicylates is characteristic (but not diagnostic) leukocytosis and normochromic anemia may
of RF. appear. Slight proteinuria and microhematuria
are occasionally seen and do not necessarily
With respect to arthritis, the dictum "one major and indicate concomitant glomerulonephritis.
2 minor criteria" is a source of diagnostic 2. As evidence of antecedent beta-hemolytic
confusion. Arthritis and arthralgia are common in streptococcal infection-A high titer or
children and young adults and are often increasing antistreptolysin O titer (ASOT)
accompanied by fever and an increased ESR. indicates recent infection but does not mean
Streptococcal infection or "sore throat" is also that RF is present. Throat culture is positive
common. Coincidental association of these factors for beta-hemolytic streptococci in 50% of cases
thus often leads to an unwarranted diagnosis of of active RF. Other specific antibodies can be
RF. A definite diagnosis requires bona fide used with high specifityy like
evidence of carditis or the appearance of additional antihyalourindase, antistreptokinase, anti-
NADase (anti-DPNase), and anti-DNAse B.

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1.Valvular Heart Diseases (VHD) 7

FIGURE 1−3. Medical mnemonic for rheumatic fever is Jones Cafe Pal and flow charts for diagnosis of
rheumatic fever.

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8 1.Valvular Heart Diseases (VHD)

3. As strong evidence against the there is any tendency toward leukopenia, the
diagnosis-A low antistreptolysin O titer (50 drug should be stopped immediately.
Todd units) that does not rise on repeated C. Treatment of Streptococcal Sore-throat:
tests tends to rule out RF. A normal ESR is It has been shown that prompt therapy (within 24
rare in the presence of active RF. hours) of streptococcal infections will prevent most
attacks of acute RF.
Differential Diagnosis § Secondary prevention: The principles of
RF may be confused with the following: rheumatoid prevention are to avoid beta-hemolytic
arthritis, osteomyelitis, traumatic joint disease, streptococcal infections if possible and to
neurocirculatory asthenia or cardiac neurosis, treat streptococcal infections promptly and
infective endocarditis, pulmonary tuberculosis, intensively with appropriate antibiotics.
chronic meningococcemia, acute poliomyelitis, § Tertiary prevention: The purpose of
disseminated lupus erythematosus, serum tertiary prevention is to reduce the
sickness, drug sensitivity, leukemia, sickle cell progression and complications of RHD.
anemia, inactive rheumatic heart disease, This includes adequate treatment of RF in
congenital heart disease, and "surgical abdomen. the initial attack, optimum medical control of
patients with RHD and timed heart valve
Prevention of Recurrent Rheumatic surgery.
Fever
§ Primordial prevention: The idea of this Treatment
type of prevention is to avoid the A. Medical Measures:
establishment of the social and cultural The salicylates markedly reduce fever, relieve
patterns known to contribute to the incresed joint pain, and may reduce joint swelling. There is
risk streptococcal infections and RF. no evidence that they have any effect on the
§ Primary prevention: The principle of natural course of the disease. Note: The salicylates
prevention is to limit the incidence of RF. should be continued as long as necessary to relieve pain,
swelling, or fever. If withdrawal results in a. recurrence of
This is primarily achieved by controlling
symptoms, treatment should immediately be reinstituted.
group A streptococcal iinfection. a. Sodium salicylate is the most widely used
A. General Measures: Avoid contact with of this group of drugs, although aspirin must be
persons who have upper respiratory infections. used if the patient has evidence of cardiac failure.
Rheumatic fever patients do better in an equable
Large doses of aspirin may produce upper
climate, where streptococcal infections are less gastrointestinal bleeding, but the risk must be
common. borne. Maximum dose is 1-2 g every 2-4 hours
B. Prevention of Infection: Two methods of orally to allay symptoms and fever; 4-6 g/d suffices
prevention are now advocated. in most adults. In an occasional patient maximum
1. Penicillin-The preferred method of prophylaxis doses may not be completely effective. There is no
is with benzathine penicillin G (Bicillin), 1.2million evidence that intravenous administration has any
units intramuscularly every 4 weeks. Oral
advantage over the oral route. Early toxic
penicillin (200-250 thousand units daily before reactions to the salicylates include tinnitus, nausea,
breakfast may be used instead but is less reliable. and vomiting. Determination of blood salicylate
Prophylaxis is advocated especially for children levels is infrequently required unless the patient
who have had one or more acute attacks, and develops symptoms suggesting salicylate
should be given throughout the school year and poisoning. Antacids are usually given with
continued until about age 25. Adults should salicylates to reduce gastric irritation. Caution:
receive preventive therapy for about 5 years after Never use sodium salicylate or sodium bicarbonate
an attack. in patients with acute RF who have associated
2. Sulfonamides-If the patient is sensitive to cardiac failure.
penicillin, give sulfadiazine, I g daily throughout the
b. Aspirin (ASA) may be substituted for
year. sodium salicylate, with the same dosages and
Caution: Patients receiving sulfonamides should precautions.
have periodic blood counts and urinalyses. If

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1.Valvular Heart Diseases (VHD) 9

FIGURE 1−4. Treatment algorithm of RF.

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10 1.Valvular Heart Diseases (VHD)

Penicillin should be employed in the treatment at given, but it should be administered with extreme
any time during the course of the disease to care.
eradicate any existing streptococcal infection. Many cases of CHF are due to acute myocarditis.
Corticosteroids-Careful studies have shown no These often respond dramatically to corticotropin or
clear or consistent proof that cardiac damage is the corticosteroids. When sodium retaining
prevented or minimized by corticosteroids even hormonal agents are employed, vigorous sodium
when they are given early in large doses. restriction (< 200 mg daily) or thiazide drugs are
Corticosteroids are effective anti-inflammatory imperative .
agents for reversing the acute exudative phase of Pericarditis- Treat as any acute nonpurulent
RF and are probably more potent for this purpose pericarditis.
than salicylates. A short course of corticosteroids The rheumatic effusion is sterile, and antibiotics are
usually causes rapid improvement in the acute of no value.
manifestations of RF and is indicated in severe The general principles include relief of pain, by
cases. There may be prompt disappearance of opiates if necessary, and removal of fluid by
fever, malaise, tachycardia, and polyarthritis. cardiac paracentesis if tamponade develops. This
Abnormal ECG changes (prolonged P-R interval) is rarely necessary.
and ESR may return to normal limits within a week. Corticotropin and the corticosteroids, as well as
Q. What is the suggested schedule? salicylates, should be continued or started, as they
A suggested schedule, to be started as soon as seem to have a specific favorable effect in aiding
severe RF is diagnosed, is as follows: Give resorption of fluid.
prednisone, 5-10 mg orally every 6 hours for 3 Q. How to treat a patient having RF and
weeks, and then gradually withdraw over a underwent valve replacement and receiving
period of 3 weeks by reducing and then anticoagulant therapy?
discontinuing first the nighttime, then the Firstly exculde infective endocarditis and give
evening, and finally the daytime doses. In corticoids in full doses with its precautions.
severe cases the dosage should be increased, if
necessary, to levels adequate to control Prognosis
symptoms. Initial episodes of rheumatic fever last months in
children and weeks in adults. 20% of children have
B. General Measures: Bed rest should be recurrences within 5 years. Recurrences are
enforced until all signs of active RF have uncommon after 5 years of well-being and rare
disappeared. The criteria for this are as follows: after age. The immediate mortality rate is 1-2%.
return of the temperature to normal with the patient Persistent rheumatic activity with a greatly enlarged
at bed rest and without medications; normal ESR; heart, heart failure, and pericarditis indicate a poor
normal resting pulse rate (under 100 in adults); prognosis; 30% of children thus affected die within
return of ECG to normal or fixation of 10 years of the initial attack. Otherwise the
abnormalities. The patient may then be allowed up prognosis for life is good. 80% of all patients attain
slowly, but several months should elapse before adult life, and 50% of these have little if any
return to full activity unless the RF was exceedingly limitation of activity. Approximately 1/3 of young
mild. Maintain good nutrition. patients have detectable valvular damage after the
C. Treatment of Complications: initial episode, most commonly a combination of
Congestive failure- Treat as for CHF, with the MS and MR. After 10 years, 2/3 of surviving
following variations: patients will have detectable valvular disease. In
A low-sodium diet and diuretics are of particular adults, residual heart damage occurs in less than
value in promoting diuresis and treating failure in 20% and is generally less severe. MR is the
acuteRF. commonest residual, and AR is much more
Digitalis is usually not as effective in acute RF as in common than in children. The influence of steroids
most cases of CHF and may accentuate the on prognosis is as yet not known. 20% of patients
myocardial irritability, producing arrhythmias that who have chorea develop valvular deformity even
further embarrass the heart. Digitalis should be after a long latent period of apparent well-being.

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1.Valvular Heart Diseases (VHD) 11

FIGURE 1−5. Management algorithm for RF.

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12 1.Valvular Heart Diseases (VHD)

Mitral Valve Diseases (MVDs)


and in larger quantity in men than in women, in
older than in younger patients, and in patients with
Mitral Stenosis (MS) higher compared with those with lower pressure
gradients between LA and LV. The rapidity with
Definition which calcification progresses also varies
It means any mechanism that causes obstruction of considerably. The primary orifice, located at the
the blood flow through the mitral valve leading to level of the annulus, is far less narrowed. Fusion
left atrial hypertension. may involve one or both commissures, when only
one is fused or one is fused more than the other,
General Pathologic Considerations the stenotic orifice is eccentrically located. A
Incidence: Over 75% of patients with MS are centrally located orifice indicates symmetrical
women below age 25 (in Egypt) and 45 (in commissural fusion.
Western countries).
Etiology : Isolated mitral disease causing MS Classification of subvalvular Apparatus in Rheumatic
is, with rare exception, rheumatic in origin, but MS (4 different stages): (Tendolkar & Parikh, 1988).
isolated mitral disease causing pure MR is usually • Type I: The architecture is nearly normal.
non rheumatic in origin. • Type II: Chordae are thickened and reduced in
A. Rheumatic MS. number, with three to four on each head of the
B. Non-rheumatic MS: They include papillary muscle. The papillary muscle. The
congenital anomalies, neoplasms (particularly papillary muscles are either fused or free (Fig.
myxoma), also malignant neoplasms, primary or 11-A).
secondary in the lung may grow into mitral orifice • Type III: There are one to two short (less than 5
by way of the pulmonary veins, large vegetations mm in length), stumpy chordae. The papillary
from active infective endocarditis , and massive muscles are always fused (Fig. 11-B).
annular calcific deposits, a relatively new, acquired • Type IV: The chordae are absent and the
cause of MS is a mechanical prosthetic or papillary muscles are directly fused with the
bioprosthetic cardiac valve. cusps (Fig. 11-C)

Rheumatic MS:
The chordae tendineae are occasionally so Complications: =Indications for surgery
retracted that the leaflets appear to insert directly 1. CHF: due to valve obstruction and
into the papillary muscles, when this occurs the MS pulmonary congestion
is always severe because the interchordal spaces 2. Infective endocarditis.
are almost entirely obliterated. Sometimes chordae 3. Systemic embolization to brain or to
inserting into one papillary muscle are well peripheral artery.
preserved, whereas those inserting into the other 4. Supraventricular tachyarrhythmias such as
papillary muscle are partially or completely fused. rapid atrial fibrillation (AF). Natural history
Normally there are about 120 third order chordae, studies now suggest that AF per se has
and about 24 first order chordae, in MS these effect on long-term survival regardless of its
numbers are usually halved. etiology.
The leaflets are diffusely thickened in all patients 5. Pulmonary hypertension:
with rheumatic MS, either by fibrous tissue or 6. Giant left atrium ( GLA). A giant LA in CXR
calcific deposits or both, the commissures are is usually a feature of MR than MS, see MR.
usually fused, and the chordae tendineae are 7. Pressure symptoms e.g. Ortner’s syndrome
shortened and usually fused to some degree. The (Horseness of voice)
greatest obstruction to this funnel shaped valve
occurs at its apex, which is within the LV cavity.
The amount of calcium in the leaflets of MS varies
considerably. Generally, calcium is more frequent

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1.Valvular Heart Diseases (VHD) 13

FIGURE 1−6. Mitral Stenosis. 2017 - The Calgary Guide to Understanding Disease. www.calgaryguide.com

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14 1.Valvular Heart Diseases (VHD)

Clinical Picture & Diagnosis Severe exertion, during pregnancy, labor, or sexual
A. Symptoms & Signs: The history will have intercourse, (2) With the occurrence of pulmonary
one or more congestive, dysrhythmia or embolic infections, (3) With the onst of AF, (4) Anemia, and
symptoms. Physical examination shows the typical (5) Fliud or blood transfusion and corticoids.
findings of localized, diastolic a low-pitched A. CXR Findings: The chest film shows 10
delayed diastolic rumble (whose duration varies classic CXR signs:
with the severity of the MS and the heart rate and • Enlargement of the left atrium: (a) As a bulge on
opening snap (because it is thickened, the valve the L. cardiac border (formed by the L. atrial
opens in early diastole with a snap with presystolic appendage), (b) As a double shadow on the R.,
accentuation if the patient is in normal sinus (c) The barium-filled esophagus is displaced
rhythm; a loud pulmonary second sound indicates backward, and (d) The L.main bronchus is
pulmonary hypertension.The sound is sharp, is elevated.
widely distributed over the chest. and occurs early • Enlargment of the pulmonary trunk.
after A2 in severe and later in milder varieties of • Small size of the aorta.
MS. If the patient has severe MS with a poor • Characteristic pulmonary vascular pattern.”antler
cardiac output and slow flow across the mitral sign or unturned moustache”. Greater vascularity
valve, the murmur may be absent or extremely in the UPPER zones than in the lower zones,
difficult to find, but the opening snap can usually be due to redistribution of pulmonary blood flow.
heard unless the patient has a grossly calcified • Parenchymal lung changes: (a) Kerley’s B lines =
mitral valve, in which case neither an opening snap B lines = Septal lines in lung bases =
nor a murmur can be heard. 50-80% of patients costophrenic septal lines = intrapulmonary septal
decelop paroxysmal or chronic atrial fibrillation (AF) lines, (b) Localized nodular shadows ( usually
that until the ventricular rate is controlled, may diffuse and attributed to hemosiderosis) =diffuse
precipitate dyspnea or pulmonary edema. 20 to reticular pattern, (c) Thickened interlobar fissures
30% of these patients in turn will later have major + effusion, and (d) Kerley’s A lines (peribronchial
emboli in the cerebral, visceral, or peripheral and perivascular cuffings).
arteries as a consequence of thrombus formation in • Mitral valvular calcification.
the LA. • RV enlargement.
In a few patients, for unknown reasons, the • RA enlargement.
pulmonary arterioles become narrowed or • LA calcification.
constricted; this greatly increases the PAP and the
• Absence of changes in LV contour.
PVR and accelerates the development of RVH and B. ECG Findings: It shows LA enlargement (P-
RV failure. These patients have relatively little mitral) and RVH. Paroxysmal or chronic AF
dyspnea but experience great fatigue and
(50-80%) with course fibrillations.
weakness on exertion because of the markedly
C. ECHO Findings: Two-dimensional
reduced cardiac output. echocarcliography is very specific in the
diagnosis of MS and provides information
Q. What is the incidence of paroxysmal related to leaflet thickness, subvalvular
nocturnal dyspnea in MS? 5-10% mechanism, degree of calcification, and ability
Q. Whart are the natural protective mechanisms to predict the possibility of surgery without
against pulmonary edemai n MS? catheterization. It shows a decreased closing
MS is a gradually occurring process, so it gives slope of the AML in mid diastole, with
time for thickening of walls of pulmonary veins, increased reflectance due to thickness of the
thus preventing trnsdudation through their walls, leaflet and chordae. The AML and PML are
(2) Development of interstitial pulmonary barrier or fixed and move together, rather than in
pulmonary fibrosis due to chronic venous opposite directions as is normal. MVA and
congestion, and (3) Development of valve score (4x4).
vasoconstrictive pulmonary hypertension.
However, acute pulmonary edema in cases of MS
may be precipitatedby the following conditions: (1)

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1.Valvular Heart Diseases (VHD) 15

FIGURE 1−7. MVD with group 2 PAH. Modified from Alhabeeb Waleed et al. Saudi Guidelines on the Diagnosis and
Treatment of Pulmonary Hypertension: Pulmonary hypertension due to left heart disease. Saudi H J. 2014 ; 9 (5) 47-55

TABLE 1−1. Diagnosis of mitral valve disease.


MS MR
Sex Females > males Males > females
History Latent interval of a few years. No
Severity of RF Less severe Often fulminating
Presystolic murmur S4 Present Absent
S1 Loud unless calcification Never loud
Apical systolic murmur Usually absent Pansystolic or late
Middiastolic murmur Long, not necessarily loud If present, short
Opening snap (OS) Present, unless heavy calcification, Rarely present
pulmonary hypertension, or AR.
S3 Never present Commonly present and loud
Cardiac impulse Tapping (“closing snap”), RV type if LV type; RV type if PVR raised
PVR raised
Radial pulse Small volume Small volume but collapsing
Systemic emboli Common Less common
LA curve Enlarged but rarely aneurysmal May be aneurysmal; systolic
LV Normal or poor filling, aorta Enlarged, rapidly filling, and
hypoplastic hyperdynamic
ECG RVH if PVR raised LVH; RVH if PVR raised
Hemodynamic data LAP may be greatly raised Less severely raised as a rule
Gradient across valve in diastole No gradient usually
PVR may be severely raised PVR not commonly greatly raised
From Orams: Clinical Heart Disease. London, William Heinenman MedicalBooks, 1971, p348.

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16 1.Valvular Heart Diseases (VHD)

D. ANGIO Findings: Cardiac catheterization is • Uncontrollable pulmonary edema. Emergency


considered for every patient with MS, but in valvotomy may be required if pulmonary edema
reality many do not require. Catheterization is develops during pregnancy.
now used primarily to diagnose associated • Disabling dyspnea and occasionally pulmonary
CAD and should be done if the patient is >40 edema When an episode of pulmonary edema
years of age or has had any history of classic has developed without any precipitating cause..
MS. Patients <40 years of age and selected • Evidence of pulmonary hypertension with right
older patients who have classic MS and no ventricular hypertrophy and early congestive
objective evidence of CAD need only failure.
diagnostic echocardiography. • Increased pulmonary arteriolar resistance, with
marked dyspnea and increased P2. These
patients are apt to develop right heart failure and
Treatment emboli.
A. Medical: • Right heart failure or tricuspid incompetence (or
Prevention: both) when secondary to marked mitral valve
1. Recurrences of acute RF can be prevented by disease.
(1) avoiding exposure to streptococcal infections', • When AF has caused deterioration and
(2) continuous antibiotic prophylaxis in selected cardioversion has failed.
patients under 35 (those with acute RF in the • Following an embolus.
preceding 5 years) and those who have been
exposed to known hemolytic streptococcal Surgical strategies:
infections, and (3) prompt and adequate • Closed mitral valvotomy (CMV): In selecting
treatment of infections due to hemolytic patients for valvotomy, thses factors are
streptococci. important: (1) The symptoms must be significant,
2. The patient should be given advice regarding (2) There must be signs of mobile valve
dental extraction, urologic procedures, surgical (presence of OS and loud S1) (3) Absence of
procedures, etc to prevent bacteremia and MR, (4) Absence of calcium on x-ray screening,
possible infective endocarditis. (5) Mobile thin cusps on ECHO.Two techniques
General Measures: Vocational guidance is could be performed:
necessary to anticipate possible reduced exercise • Transventricular approach: Using Tubbs-
tolerance in late life. Follow-up observations should Logan dilator, the most common technique.
emphasize early recognition of disturbances of • Transatrial approach: Using Dubost dilator,
thyroid function, anemia, and dysrrythmias; considered to be obslete.
maintenance of general health; and avoidance of
• Open mitral valvuloplasty (OMC): In patients
obesity and excessive physical exertion.
who have no to mild calcification with pure MS,
B. Interventional: For valve obstruction < 1.5
there is a > 95% probability of an open mitral
cm2 with symptoms shown either by noninvasive
valvuloplasty. This technique (Fig. 1) involves
studies or by direct measurement at cardiac
incision of both commissures, incision of fused
catheterization or balloon-dilatation is
chordae, and occasionally incision into the
appropriate.
papillary muscle to increase mobility where there
C. Surgical: Indications for surgery:
has been shortening of the chordae due to
Because the course of MS is highly variable and
scarring and fibrosis.
because of the significant mortality rate (3-5%) as
• Mitral valve replacement (MVR): Moderate to
well as morbidity associated with mitral valvulotomy
severe calcification suggests that valve
and the frequency of restenosis, surgery is not
replacement will be necessary. The calcification
advised in mild cases with slight exertional
may extend down onto the subvalvular
dyspnea and fatigue only. Indications for surgery
structures, obliterating the subvalvar spaces
include the following:
between chordae tendineae, and sometimes
• Signs of mitral stenosis with a pliable valve
may involve the tips of the papillary muscles. The
(opening snap, snapping first sound).
technique of annulo-papillary continuity could be
used with good results in MS.

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1.Valvular Heart Diseases (VHD) 17

FIGURE 1−8. Diagnosis & management algorithm for rheumatic MS. Modified from MA Papadakis, SJ McPhee, MW
Rabow: Current Medical Diagnosis & Treatment 2017, 56th Ed. McGraw-Hill Education.

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18 1.Valvular Heart Diseases (VHD)

1. MVR and left atrial aneurysmorraphy. A. Rheumatic: Why the mitral valve affected
2. MVR and MazeIII or its modifications by acute RF becomes stenotic in some patients
operation with different power sources and purely reguragitant in others (mainly children),
(see surgery for AF). is unknown. Although the cause is similar, the
Prognosis structural alterations in the purely regurgitant mitral
With good leaflet pliability and subvalvular valve are quite different from those in the stenotic
mechanism, percutaneous transatrial balloon valve. The purely regurgitant rheumatic valve
dilatation is popular at present, although the long- infrequently contains calcific deposits, its
term results are not yet known. commissures are virtually never fused, and its
Q. What are the causes of recurrent symptoms chordae tendineae are only mildly thickened, if at
after mitral commissurotomy? MITRTAL all, chordal fusion is infrequent. It is often difficult to
Restenosis. This needs 3 satisfactory explain adequately the mechanism of MR in a
hemodynamic studies (one preoperative and 2 valve made purely regurgitant by consequences of
postoperative), (2) The presence of MR or RF.
development of MR (either at the operation or as In RF, the inflammatory process and the
consequence of infective endocarditis or late subsequent fibrosis is very often concentrated at
calcification), (3) Progression of Aortic valve the free edges of the valve leaflets, particularly at
disease (may be mild and increase or hidden or the points of insertion of the chordae tendineae. As
missed), (4) The development of Ischemic heart the depth of the PML is half that of the AML,
diseas, (5) Myocardial factor ( rheumatic equivalent contraction of the scar tissue in both
cardiomyopathy), and (6) Recurrent attacks of leaflets will adversely affect the function of the PML
bronchitis and COPD (Lung diease). before that of the AML. This explains WHY?
Q. What are the determinant of risk factors for shortening of PML is the most common cause of
MVR in MS and the incidence of LV rupture? rheumatic MR.
Papillary muscle dysfunction or necrosis
following acute myocardial infarction (PMI-MR)
Mitral Regurgitation (MR) is less common. When the MR is due to papillary
dysfunction, it may subside as the infarction heals.
Other causes include cardiac tumors, especially left
Definition atrial myxoma, and surgically acquired mitral
The mitral leaflets do not close normally during insufficiency.,
ventricular systole, and blood is forced back into
the LA as well as through the aortic valve. The net B. Non-rheumatic (= Diseases of the Mitral
effect is an increased volume of work by the LV. Complex: Have different clinical findings and a
different clinical course)

General Pathologic Considerations Carpentier Classification


Incidence: Valve analysis is simplified with the functional
Etiology : Isolated mitral disease causing MS is, approach, since it is necessary only to determine
with rare exception, rheumatic in origin, but isolated whether the motion of each leaflet is normal (Type
mitral disease causing pure MR is usually non I), prolapsed (Type II), or restricted (Type III).
rheumatic in origin. This classification is helpful in recognizing the
Chronic MR lesions that produce this dysfunction (Table IV).
Rheumatic MR. Thus prolapsed leaflet may result from chordal
Non-rheumatic MR: The diverse etiologies include rupture or elongation or from papillary muscle
coronary disease, infection, trauma, and rupture or elongation. Restricted leaflet motion may
congenital anomalies. The most common cause in result from commissural fusion, leaflet thickening,
North America is mitral valve prolapse (MVP). chordal fusion and/or chordal thickening. Several
Acute MR lesions are usually associated. When the motion of
the two leaflets is normal, mitral valve
In contrast to MS, which has mainly one cause incompetence may be due to leaflet perforation or
(rheumatic), pure MR has numerous causes: pure annular dilation.

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1.Valvular Heart Diseases (VHD) 19

TABLE 1−2. Acute vs. chronic severe MR.


Chronic MR Acute MR
Mechanisms RF, MVP, CAD, LV dilatation, Calcified Rupure of chordae (IE, MVP, trauma &
mitral annulus, Connective tissue spontaneos), Rupture of papillary muscle
disorders, Papillary muscle dysfunction, or (PMI-MR), Perforation of leaflet (IE)
Congenital MR
Sex Both men & women More in men
Age at onset Adolescence & young adulthood Usually >45 years old
History of murmur Many years Recent onset
Natural history CHF occurs after long history of murmur CHF begins with onset of murmur
Rhythm AF (usual); SR (less common) SR (usual)
Murmur Holosystolic, blowing apical and radiating Holosystolic, loud, rough and decreasing
towards the axilla in late systole; often radiating to base;
confused with AS
S3 Present Present
S4 Absent (usually) Present
LA size Enlarged, sometimes huge Normal or mildly enlarged
Pulmonary hypertension Mild to severe Mild to severe
Ronan J, Steelman R, Deleon A, Waters T, Perloff J, Harvey W. The diagnosis of acute severe mitral insufficiency. Am J Cardiol
1971;27:283

TABLE 1−3. Rheumatic vs. non-rheumatic MR


Rheumatic MR Nonrheumatic MR
Gender More in females More in males
CHF Usually slowly usually more rapidly
Course Develops over a period of many years. More acute and patients get into trouble
within months or 1-2 years.
Rhythm Usually in atrial fibrillation than in SR In SR rather than AFib,
LAE Usually present Little or no
Calcification Yes No
Associated MS Yes No
ECHO and ANGIO appearence Diagnostic Diagnostic and often present an ANGIO
appearance that may be helpful.
Prognosis without surgery Less fulminant more fulminant
Surgical treatment (repair vs. MVR) Elective surgery is usually required Urgent surgery is usually required.

FIGURE 1−9. Diagnosis and


management algorithm for MR.. A
and P indicate projections of anterior
and posterior leaflets onto the mitral
annulus; C; mitral leaflet coaptation
height; MR, mitral regurgitation; MSD,
minimal systolic displacement; and
TGF-β, transforming growth factor-β.
Francesca N. Delling, and Ramachandran
S. Vasan. Epidemiology and
Pathophysiology of Mitral Valve
Prolapse. Circulation. 2014;129:2158-
2170

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20 1.Valvular Heart Diseases (VHD)

Complications size, thus reducing the area that the leaflets are
• CHF required to bridge. Even so, the surface area of
• Pulmonary hypertension the leaflets is about two and one half times, the
• MR, like MS, predisposes to atrial fibrillation; but area of the orifice, thus providing a comfortable
this arrhythmia is less likely to provoke acute reserve.
pulmonary congestion, and fewer than 5% of
patients have peripheral arterial emboli. 3- Left Atrial Wall
• MR especially predisposes to infective Two contributions of the LA have been related to
endocarditis. competence of the mitral valve: (1) contraction and
• Giant LA leading to 4 complications: Normally relaxation, (2) Atrial dilatation. Contractility rather
the LA is the most superior and most posterior of than flow has been considered the chief
the cardiac chambers lying just under the determinant of mitral valve closure as a result of
tracheobronchial bifurcation in front of the spine atrial activity. Although atrial contraction and
with the descending aorta and esophagus in relaxtion seem capable of closing the mitral valve
between. It is partially bounded anteriorly by the in man, the absence of such activity does not
great vessels arising from the base of the heart, cause MR LA enlargement itself can contribute to
the LV lies to the left, anteriorly and inferiorly. MR.
The four pulmonary veins enter the LA at the Dilatation of the LA does not affect the AML, since
comers of its posterior wall which is that leaflet is anchored to the root of the aorta. The
quadrangular in outline. The R. and L. PML, however can be directly affected. As the LA
pulmonary arteries run along its upper border. enlarges, its posterior wall is displaced posteriorly
The LA can enlarge in the pathway of least and downward. Because of the continuity of the
resistance, laterally to the R. or L., superiorly atrial endocardium and PML, this displacement
displacing the adjacent bronchi with widening of exerts tension on the PML. The displacement may
the carinal angle, posteriorly displacing the prevent that cusp from contraction, its mate or may
esophagus. It can enlarge anteriorly and slide aggravate preexisting leaflet malapposition. Thus,
behind the base of the LV bending its as MR provokes LA enlargement, the enlargement
posterobasal wall. It can obstruct the venous itself may aggravate regurgitant flow.,” MR begets
return at the IVC arifice by marked displacement MR”, irrespective of the initiating cause.
of the atrial septum.
2- Mitral Annulus
The mitral apparatus is a complex, finely A traditional point of view states that, if the AV ring
coordinated mechanism that requires for its dilates sufficiently, the cusps are unable to meet
competence, the functional intergrity of six and MR results. This explanation is an over
anatomic elements working in delicate concert. The simplification. The sphincter – like contraction of
exact mode of closure of the normal mitral valve is the annulus during systole, reduces the area that
still uncertain, left ventricular systole begins with the apposing leaflets must bridge by an estimated
contraction of the papillary muscles, the vertical 20 to 50%. LV dilatation may exert an unfavorable
forces exerted by the contracting papillary muscles effect on the annulus chiefly by applying a
move the leaflets into apposition. Disease involving distending pressure that opposes systolic annular
these parts results in reduced leafl et coaptation contraction. With calcification of the annulus, in
leading to MR, described using Carpentier’s itself a cause of MR the mechanism of MR is
pathophysiological triad (TABLE 1- ) and believed to stem a loss of sphincteric action of the
segmental valve nomenclature. basal attachments of the mitral cusps.

As the intra-ventricular pressure rises, the free 3- Leaflets


edges of the cusps firmly coapt, mutually Proper closure of the leaflets represents an
supporting each other along a comfortable margin ultimate goal of the entire mitral mechanism.
of their atrial surface and firmly sealing the orifice. Consequently, the AML is more mobile, while the
The remainder of each leaflet bulges like a PML fulfills a secondary or supporting role.
parachute towards the LA. The annulus not only
serves as a fulcrum for the leaflets, but during
ventricular systole decreases its circumferential

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1.Valvular Heart Diseases (VHD) 21

FIGURE 1−10. Carpentier’s pathophysiological triad and segmental valve nomenclature.

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22 1.Valvular Heart Diseases (VHD)

Although the mitral leaflets, like other cardiac abnormally during ventricular systole and focal
valves, have been looked upon as passive fibrous thickening results. Since the LV cavity
connective tissue structures, recent investigations diminishes with age, the mitral annulus does also.
have found within their substance striated muscle This scarring from aging, however, is not enough to
bundles to which an active function has been cause mitral dysfunction.
assigned. It is uncertain whether such muscle Q. What are the differences between MS & MR in
fibres have physiologoic significance in promoting symptomatology?
closure of the valve. Symptoms of MR are similsr to MS except that:
Symptoms of low cardoaic output (chronic
4- Chordae Tendineae weakness and fatigue) can preceed symptoms of
The etiology of spontaneous chordal rupture is pulmonary congestion (dyspnea, cough, and
often unclear. Infective endocarditis is one hemoptysis).
established cause, but rupture of chordae Cardiac asthma and acute pulmonary edema are
tendineae is not a complication of AMI. Rupture of LESS common (because the sudden increase of
one or more chordae results in acute loss of leaflet LAP is less common) and tend to occur LATELY
support and in the abrupt onset of appreciable MR, (due to LV failure).
the key to the physiologic disturbance and clinical The most common complaint in MR IS palpitation
picture is the sudden development of severe MR (due to hyperdynamic LV and frequent PVCs).
into a previously normal, small and therefore Systemic thromboembolic manifestations and
relatively non-compliant LA. pulmonary hypertension are LESS often-and atrial
fibrillation more often than do patients with MS.
5, 6 Papillary Muscles and Left Ventricular Wall N.B.: Because of the frequency of combined MS
These two elements represent the muscular and MR, and because of the difficulty in some
components of the mitral apparatus. The papillary patients with fixed valves to specify which is
muscles as a functional unit, includes a portion of dominant, dogmatic differentiation is unwise.
the adjacent LV wall. Papillary muscle dysfunction When MR is combined with AS or AR, patients
without loss of continuity can result in either
may become symptomatic with lesser
persistent MR of varying severity, or in intermittent
hemodynamic abnormalities of both valves than
MR during episodes of ischemia (angina) without
infarction. if the valves were diseased individually

Q. How might LV enlargement render the Clinical Picture & Diagnosis


mitral valve incompetent? B. Symptoms & Signs: The history and physical
The effect of altered LV shape on the position of examination substantiate the etiology, and the
the papillary muscles and their directional axes of physical examination documents enlargement of
tension. When the papillary muscles are not the heart and harsh, holosystolic apical murmur
vertically aligned with the annulus (lateral migration
due to spherical dilatation of the LV)., the systolic TABLE 1−3. The essential diagnostic signs of
forces exerted on the leaflets via the chordae are in uncomlicated cases of MR
a lateral as opposed to a vertical direction. This Left ventricular VOLUME overrload:
lateral tension, especially on the AML, opposes • Hyperdynamic apex beat displaced out and
apposition and renders the valve incompetent. downwards.
Dilatation of the annulus, but this appears to be REGURGITATION through the mitral valve:
inadequate even though admittedlly the mitral • Systolic thrill at the apex
orifice can dilate. In part, dilatation may prevent • Pansystolic murmur propagated to the axilla.
incompetence. In part, dilatation may prevent the • Faint S1
annulus from decreasing its circumferential size Rapid FLOW OF BLOOD across the mitral valve:
during ventricular systole. • S3
Aging. Generally, with aging, the mitral valve, like
• Short middiastolic murmur ( in severe cases
other tissues changes. This change consists mainly
ONLY).
of focal fibrous thickening of both leaflets and
chordae tendineae. The LV cavity becomes
smaller. The leaflets then contact one another

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1.Valvular Heart Diseases (VHD) 23

FIGURE 1−11. Algorithm for


distinguishing severe from
nonsevere MR in patients with
clinically significant MR jets on
color Doppler imaging Modified
from Grayburn, PA et al. Quantitation
of Mitral Regurgitation.
Circulation. 2012;126:2005-
2017.Zoghbi WA, et al.
Recommendations for evaluation of
native valvular regurgitation with two-
dimentional and Doppler
echocardiography, J Am Soc
Echocardiogr 2003;16(7):777.

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24 1.Valvular Heart Diseases (VHD)

Q. What is the classification of grades of MR? noninvasive testing should. proceed to cardiac
In patients with chronic MR, the MR has widely catheterization and coronary y angiography if they
diverse etiologies. Patients generally present in are >40 years old or have chest pain syndrome.
two categories: Symptomatic patients with CHF, FC III and IV, who
have documented MR on ECHO should progress
TABLE 1−4. Clinical grading of MR immediately to cardiac catheterization and
Uncomplicated cases: those with a murmur and coronary angiography.
relatively asymptomatic, functional class (FC) I or II For the indications see TABLE 2- for ACC/AHA
Complicated cases: those with CHF from longstanding task force guidelines on coronary angiography in
MR, who are usually very ill in FC III or IV. patients with valvular heart disease.
• Complicated cases with LV failure with signs of
pulmonary congestion. Treatment
• Complicated cases of pulmonary hypertension. G. Medical: If a person has a murmur but
• Complicated cases with superadded R. sided normal heart size on noninvasive testing, no further
CHF. therapy is indicated. The patient should be followed
at 6-month intervals with two- echocardiography.
C. CXR Findings: It establishes cardiomegaly. The results of these studies suggest that in patients
The serial CXR examination, particularly in with mild to moderate MR, with or without general
asymtomatic patients, determines whether there hypokinesis of the ventricle and ejection fraction
has left progressive enlargement of the heart over (EF) <0.20, valve surgery is not indicated and
time. medical therapy alone will suffice if they are >65
D. ECG Findings: It establishes cardiomegaly years of age. Medical treatment can be used for
(LVH). minimal LV failure or arrhythmias if the lesion is
E. ECHO Findings: 2D is a major noninvasive considered sufficiently mild not to warrant cardiac
diagnostic aid. In patients who are asymptomatic surgery. Vasodilator therapy has proved a useful
or, at best, FC II, the decision for surgical therapy addition to the management of CHF in MR.
depends on documentation of enlarging heart size H. Interventional: § see PMVR.
and progressive LV dysfunction, usually I. Surgical:
determined by serial ECHO determinations of • Patients with 3+ or 4+ MR + EF >0.20 èMV
systolic, and diastolic volume indices. It is valuable surgery. The mitral valve operation of choice
in demonstrating disruption of chordal support of depends on the physical characteristics of the
the mitral valve with MVP (1 or 2); the diagnostic valve at the time of surgery.
finding is posterior MVP in systole, usually o A heavily calcified annulus and/or valve è
associated with early diastolic anterior Motion of MVR (prosthetic or bioprosthetic valve).
the PML. This is to be contrasted with the ECHO o A dilated annulus, + pathology as ruptured
findings of the midsystolic click, late systolic chordae, prolapsed leaflets, or annular
murmur syndrome (Barlow's syndrome), in which dilatation, è MV repair by standard repair
there is late systolic MVP (notching of the posterior techniques (FIGURE 1-.)
MVL) without the diagnostic early anterior motion • MVD + GLA èMVR and LA aneurysmorraphy
seen in chordal rupture. LA dimension is obtained (FIGURE 1-.)
in the supine position by standard LPLAX view with • MVD + AFib èMVR and Maze III or IV operation
2D and M-Mode ECHO at the time of aortic valve or electrocautary maze § see after.
closure and is averaged over 3 consecutive beats. • Combind MVD (rheumatic or nonrheumatic) +
Quantification of MR (see after, FIGURE 1- ). CADè MVR and CABG (FIGURE 1- ) § see
Proposed temporal spectrum of MVP progression after.
with potential interventions/nonsurgical therapies • MVD + <65yr + EF <0.20 è orthotopic heart
depending on progression stage (bottom). FIGURE transplantation (OHTx) § see vol V.
1- • MVPèThe management strategies of MR due to
MVP pregnant patient. (FIGURE 1- )
F. ANGIO Findings: Patients with an enlarged
heart on CXR studies and LV dysfunction on

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1.Valvular Heart Diseases (VHD) 25

FIGURE 1−12. Diagnosis and management algorithm for MR. Modified from MA Papadakis, SJ McPhee, MW Rabow:
Current Medical Diagnosis & Treatment 2017, 56th Ed. McGraw-Hill Education.

TABLE 1−5. ACC/AHA task force guidelines on coronary angiography in patients with valvular heart disease
Indication Class
1. Before valve surgery (including infective endocarditis) or mitral balloon commissurotomy in patients with: I
a. Chest pain
b. Other objective evidence of ischemia
c. Decreased left ventricular systolic function
d. History of coronary artery disease
e. Coronary risk factors (including advanced age)
2. Patients with apparently mild to moderate valvular heart disease but with: I
a. Progressive (class II or greater) angina
b. Objective evidence of ischemia
c. Decreased left ventricular systolic function
d. Overt congestive heart failure
3. Before valve surgery in men aged 35 and older, premenopausal women aged 35 years or older who have coronary risk factors, I
and postmenopausal women
4. Surgery without coronary angiography is reasonable for patients having emergency valve surgery for acute valve regurgitation, IIA
aortic root disease, or infective endocarditis
5. Patients undergoing catheterization to confirm the severity of valve lesions before valve surgery without pre-existing evidence of IIB
coronary artery disease,multiple coronary risk factors, or advanced age
6. Coronary angiography is not indicated in young patients undergoing nonemergent valve surgery when no further hemodynamic III
assessment by catheterization is deemed necessary and no coronary risk factors, no history of coronary artery disease, and no
evidence of ischemia are present
7. Patients should not undergo coronary angiography before valve surgery if they are severely hemodynamically unstable III
Bonow RO, Carabello BA, Chatterjee K, et al: ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American
College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients
with Valvular Heart Disease). Available at: http://www.americanheart.org.

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26 1.Valvular Heart Diseases (VHD)

Prognosis the overall population and for each subset. Subset


The early and late embolic complications of MR models were developed for isolated AVR, isolated
must be recognized, but the surgical mortality rate MVR, multiple valve replacement, AVR with CABG,
has declined with greater experience. The fate of MVR with CABG, multiple valve replacement with
the prosthesis over the long-term is uncertain. CABG, and valve replacement with other thoracic
Follow-up studies have shown considerable clinical aortic surgery.
and hemodynamic improvement in surviving The database contains complete records of 86,680
patients, including reversal of pulmonary and left patients who had valve replacement procedures at
atrial hypertension. Usually, however, some the participating institutions between 1986 and
hemodynamic abnormality persists. 1995, inclusive. The 1995 harvest of data was
Operative mortality includes any death occurring conducted in late 1996 and available for evaluation
within 30 days after the operation or within the in 1997. These records were used to conduct an
same hospital admission. in-depth analysis of risk factors associated with
Late cardiac death is defined as a terminal event valve replacement and to provide prediction of
attributed to cardiaccauses taking place after operative death by using regression analysis.
patient discharge from the hospital (more than 30 Regression models were made for six subgroups.
days after the operation).
Predictors of survival
The operative mortality rates for cardiac valve • Left ventricular E.F: E.F. measured by
replacement procedures have been reported from echocardiography is the most powerful predictor
1 % to 15% depending upon valve position, of late survival. Surgical treatment should be
multiple replacement, and concomitant CABG. The considered early (before the E.F. decreases to
quality assurance of valve replacement operations 50%), even in the absence of severe symptoms
can be determined only by assessing the in patients with severe M.R. before LV
relationship between preoperative risk factors and dysfunction occurs. It has been reported that
operative mortality. Several reports have when E.F. is >70% a favorable surgical outcome
documented variables, either preoperative or is predicted and when it is <55% a poor outcome
intraoperative, that are independently related to is predicted. An E.F. <40% in patients with
increased risk of operative mortality. The Veterans severe MR usually represent advanced
Administration study of preoperative risk myocardial dysfunction, such patients are high
assessment for cardiac operations proposed that operative risks and may not experience marked
the ratio of observed to expected mortality is a improvement following MVR.
superior method to assess quality of care than • Preoperative end-systolic diameter (ESD): If it
unadjusted mortality. That study found, by > 50mm, a poor postoperative outcome is
multivariate logistic regression analysis, that priority predicted despite chordal preservation in patients
of reoperation, age, peripheral vascular disease, with M.R. and when preoperative ESD is 40mm
mitral valve replacement, and cardiomegaly were or less an excellent outcome is predicted
independent predictors of death for patients who • Preoperative pulmonary hypertension (mean
had cardiac valve operations with or without PAP >20mmHg) correlates with persistent
coronary artery bypass grafting. postoperative L.V. dilatation.
The Society of Thoracic Surgeons (STS) National
Cardiac Surgery Database has been used to
conduct a detailed analysis of risk factors
associated with valve replacement operations to
predict operative mortality. In the evaluation we
paid particular attention to the influence of valve
position, urgency, concomitant coronary artery
bypass, and reoperation. The previous studies that
used the STS database primarily documented risk
stratification related to myocardial revascularization
The risk stratification modeling evaluated the
influence of 51 preoperative variables on operative
mortality by univariate and multivariate analysis for

Ezzeldin Mostafa, Perspectives in Cardiovascular & Thoracic Surgery © 2017, Elnasr Publishing Co.
All rights reserved. Usage subject to terms and conditions of license.
1.Valvular Heart Diseases (VHD) 27

FIGURE 1−13. Algorithm for management during minimally invasive mitral valve surgery. Kalavrouziotis, D;
Dagenais, F. 54: Minimally Invasive Cardiac Surgery. In David D. Yuh, Luca A. Vricella, Stephen C. Yang, “John R. Johns
Hopkins Textbook of Cardiothoracic Surgery” › 2014, 2e.

Ezzeldin Mostafa, Perspectives in Cardiovascular & Thoracic Surgery © 2017, Elnasr Publishing Co.
All rights reserved. Usage subject to terms and conditions of license.
1.Valvular Heart Diseases (VHD) 111

FIGURE 1−63. Ventriculoaortic junction (VAJ) and 3 techniques of root enlargement. Modified from Ann Thorac
Surg 1984;38:76.

Ezzeldin Mostafa, Perspectives in Cardiovascular & Thoracic Surgery © 2017, Elnasr Publishing Co.
All rights reserved. Usage subject to terms and conditions of license.

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