A Quick Tour Of Pediatric

Cardiology

LIVINGSTONE
CONGENITAL HEART
DISEASES
Introduction

Most common category of congenital

structural malformation
Commonly divided into noncyanotic (L 

R) and cyanotic (R  L) categories based

on direction of shunting
Relative Frequency of Lesions
 Ventricular septal defect 25-30
 Atrial septal defect (secundum) 6-8
 Patent ductus arteriosus 6-8
 Coarctation of aorta 5-7
 Tetralogy of Fallot 5-7
 Pulmonary valve stenosis 5-7
 Aortic valve stenosis 4-7
 Transposition of great arteries 3-5
 Hypoplastic left ventricle 1-3
 Hypoplastic right ventricle 1-3
 Truncus arteriosus 1-2
 Total anomalous pulm venous return 1-2
 Tricuspid atresia 1-2
 Double-outlet right ventricle 1-2
 Others 5-10
Noncyanotic CHD (L R)
 Atrial septal defects (ASD)
 Ventricular septal defects (VSD)
 Patent ductus arteriosus (PDA)
 Obstruction to blood flow
 Pulmonic stenosis (PS)
 Aortic stenosis (AS)
 Aortic coarctation
Atrial Septal Defect
 Most commonly asymptomatic
 Essentials of diagnosis:
 Right ventricular heave
 S2 widely split and usually fixed
 Systolic murmur at the pulmonary area
 Cardiac enlargement on CXR
Atrial Septal Defect
Atrial Septal Defect
 Three major types
 Ostium secundum
 most common
 In the middle of the septum in the region of the foramen
ovale
 Ostium primum
 Low position
 Form of AV septal defect
 Sinus venosus
 Least common
 Positioned high in the atrial septum
 Frequently associated with PAPVR
Atrial Septal Defect
 Treatment
 Closure generally recommended when ratio
of pulmonary to systemic blood flow is >
2:1
 Operation performed electively between
ages 1 and 3 years
 Previously surgical; now often closed
interventionally
Atrial Septal Defect
Ventricular Septal Defect
 Single most common congenital heart
malformation, accounting for almost
30% of all CHD
 Defects can occur in both the
membranous portion of the septum
(most common) and the muscular
portion
Ventricular Septal Defect
Ventricular Septal Defect
 Three major types
 Small, hemodynamically
insignificant
 Between 80% and 85% of all VSDs
 < 3 mm in diameter
 All close spontanously
 50% by 2 years
 90% by 6 years
 10% during school years
 Muscular close sooner than
membranous
Ventricular Septal Defect
 Moderate VSDs
 3-5 mm in diameter
 Least common group of children (3-5%)
 Without evidence of CCF or pulmonary
hypertension, may be followed until
spontaneous closure occurs
Ventricular Septal Defect
 Large VSDs
 6-10 mm in diameter
 Usually requires surgery, otherwise…
 Will develop CCF and FTT by age 3-6
months
Ventricular Septal Defects
 Clinical findings
 Medium- to high-
pitched, harsh
pansystolic murmur
heard best at the left
sternal border with
radiation over the
entire precordium
Ventricular Septal Defect
 Treatment
 Indicated for closure of a VSD associated
with CCF and FTT or pulmonary
hypertension
 Patients with cardiomegaly, poor growth,
poor exercise tolerance, or other clinical
abnormalities and a P/S > 2:1 typically
undergo surgical repair at 3-6 mo
Patent Ductus Arteriosus
 Persistence of normal fetal vessel joining the
pulmonary artery to the aorta
 Closes spontaneously in normal term infants
at 3-5 days of age
 Epi facts
 Accounts for about 10% of all cases of CHD
 Higher incidence of PDA in infants born at high
altitudes (> 10,000 feet)
 More common in females
Patent Ductus Arteriosus
 Accounts for about 10% of all cases of
CHD
 Higher incidence of PDA in infants born
at high altitudes (over 10,000 feet)
 More common in females
Patent Ductus Arteriosus
Patent Ductus Arteriosus
 Clinical findings and course depend on
size of the shunt and the degree of
associated pulmonary hypertension
Patent Ductus Arteriosus
 Pulses are bounding and pulse pressure
is widened
 Characteristically has a rough
“machinery” murmur which peaks at S2
and becomes a decrescendo murmur
and fades before the S1
Patent Ductus Arteriosus
 Treatment consists of surgical correction
when the PDA is large except in patients with
pulmonary vascular obstructive disease
 Transcatheter closure of small defects has
become standard therapy
 In preterm infants indomethacin is used (80-
90% success in infants > 1200 grams)
Cyanotic CHD (R L)
 Tetralogy of Fallot (TOF)
 Tricuspid atresia (TA)
 Total anomalous pulmonary venous return
(TAPVR)
 Truncus arteriosus
 Transposition of the great vessels
 Hypoplastic left heart syndrome (HLH)
 Pulmonary atresia (PA) / critical PS
 Double outlet right ventricle (DORV)
Tetralogy of Fallot

 Most common cyanotic heart

disease.
 The four abnormalities include:
 Pulmonary stenosis
 RVH
 VSD
 Overriding Aorta
 Signs include cyanosis, murmur,
squatting and spells.
Tetralogy of Fallot
Tetralogy of Fallot
 Most common cyanotic lesion (7 to 10%
of all CHD)
 Typical features
 Cyanosis after the neonatal period
 Hypoxemic spells during infancy
 Right-sided aortic arch in 25% of all
patients
 Systlic ejection murmur at the upper LSB
Tetralogy of Fallot
 Clinical findings vary depending on
degree of RVOFT obstruction
 Most patients are cyanotic by 4 months
and it is usually progressive
 Hypoxemic spells (“tet spells”) are one
of the hallmarks of severe tetralogy
Tetralogy of Fallot
Tetralogy of Fallot
 Tet spells most commonly start
around 4 to 6 months of age and are
charcterized by
1. Sudden onset or deepening of cyanosis
2. Sudden onset of dyspnea
3. Alterations of consciousness
4. Decrease in intensity of systolic murmur
TOF cont..
 A “tet” spell consists of rapid breathing
and increased cyanosis. Any event like
crying or increased physical activity can
initiate the spell.
 Treatment includes:
 holding the baby in a knee chest position
 Morphine
 Oxygen, beta blocker, general anesthesia,
Tetralogy of Fallot
 Repair may be staged (modified BT
shunt) or complete
Transposition of the great
Arteries
 The aorta arises from the right ventricle and
the pulmonary artery from the left.
 The mixing of the blood occurs at the PFO
and the PDA.
 The signs include cyanosis and cardiomegaly.
 There may be no murmur.
 An echocardiogram is diagnostic.
COARCTATION OF THE
AORTA
Coarctation of the Aorta
(CoA)
 More common in males

 Almost always juxtaductal

 85% of children with CoA have

a bicuspid aortic valve.
 CoA - cont….
 Symptoms and Signs:
SEVERE : Shock
MODERATE : CHF,
MILD : Headaches, leg
claudication
 Decreased femoral pulses are an
important sign esp. in neonates.
 BP lower in the lower limbs
CoA - cont….
 ECHO : Diagnostic
 Treatment: For an infant in
shock -PGE1 immediately.

 Surgical vs. transcatheter

repair.
Hypoplastic Left Heart
Syndrome
 Varying degrees of left heart hypoplasia at
multiple levels
 Babies present in cardiogenic SHOCK once
the ductus closes.
 Immediate treatment is PGE1 intravenously
as an infusion.
Hypoplastic Left Heart
syndrome
SYNDROMES
 Down’s: Incidence 50% . AV canal defects.
 Turner’s : 10%. Coarctation , bicuspid aortic
valve
 Williams’s : Supravalvar aortic stenosis, PPS
 Alagille : Peripheral pulmonic stenosis (PPS)
 Noonan : PPS and HCM
 Marfan’s : Aortic root dilatation, MVP
 DiGeorge: Truncus Arteriosus, Interrupted
aortic arch.
 Catch 22 : conotruncal abn. such as VSD,TOF,

collaterals, right aortic arch

immotile cilia
Environmental Factors/Toxins

 Lithium: Ebstein’s anomaly

 Ethanol: ASD,VSD ( Fetal Alcohol
Syndrome)
 Anticonvulsants: PS, AS, TOF
 Retinoic Acid: Transposition
 Rubella: PDA, PPS
 Coxsachie B : Neonatal myocarditis
 Maternal Diabetes: HCM, TGA
 Maternal Lupus: Complete heart block
 PKU: VSD, ASD, complex CHD
Innocent Murmurs
 Diastolic murmurs are never innocent
 Innocent murmurs are present in
at least 50 % of normal children
 Still’s murmur : low pitched, vibratory,
systolic ejection, increases with the supine
position.
 Venous hum: continuous murmur in
supraclavicular region, reduces on lying
down or with pressure on neck.
RHEUMATIC FEVER
Jones’ Modified Criteria
Rheumatic Carditis
 Present in 50% cases
 “Sleeping” tachycardia is an early
sign
 Mitral and aortic valves most
commonly involved
Rheumatic Arthritis
 Most common manifestation
 Pain, swelling and erythema
 Resolves within 1 week
RF-Treatment and
Prevention
 Benzathine penicillin 1.2 mega
units IM
 Aspirin 75-100 mg/kg for 6-8
weeks
 Steroids for severe carditis
 Digoxin , diuretics
 Prevention with BP q 4 weeks.
Congestive Cardiac Failure
 Tachycardia
 Tachypnea
 Hepatomegaly
 Cardiomegaly, murmur, HR too fast/slow
 FAILURE TO THRIVE
 CHD
 2 months-VSD, PDA
 Within 1st month- Coarctation, AS, HLHS
 Neonatal period:Truncus Arteriosus
 Normal heart
 Myocarditis
SYNCOPE
 Brief loss of consciousness with rapid
recovery
 Seen in adolescents and in toddlers
 20-50% of adolescents experience at
least one episode of syncope
 most cases benign
 Vasovagal syncope is the most common
type in adolescents
 Typical history , normal EKG
BENIGN SYNCOPE
 Vasovagal

 Orthostatic hypotension

 Hyperventilation

 Breath holding spells

DANGER SYMPTOMS
 Syncope especially with
EXERTION or EXCITEMENT-
anger, fear, startle

 Cardiac arrest with exercise or

excitement
Degree Entry-Level
Competencies and
Baccalaureate Degree
Entry-Level Competencies
definitions. [8][9]