Approach to Congenital Heart

Disease

HOD and Professor of Pediatrics

 CHD – Approach
• History
• Clinical Examination
– Inspection, Palpation, Auscultation
• Investigation – ECG, X-Ray, ECHO
• Treatment
History - Congenital Heart
Diseases
 Complications of CHD
Acyanotic CHD Cyanotic CHD
• Congestive cardiac failure • Clubbing
• Pulmonary Hypertension • Cyanotic Spell
• Reversal of shunt • Depressed IQ
• Infective endocarditis • Infective endocarditis
• Polycythemia
• Embolic phenomenon
 Chief complaints
• Breathlessness
• Palpitation
• Chest pain
• Cough
• Edema
• Failure to thrive
• Joint pain / swelling
• Syncope
 History
• Cyanotic spells
• Squatting episodes
• Sore throat
• Hematuria
• Pink frothy sputum
• Convulsions
 General examination
• Anemia
• Cyanosis
• Clubbing
• Signs of infective endocarditis
• Signs of failure
• Signs of Rheumatic fever
• In Syndromes – congenital heart disease
 In Syndromes
• Downs syndrome – Endocardial cushion defect
• Rubella syndrome – PDA
• Turner’s syndrome – COA
• Trisomy 13 – VSD, ASD, PDA, Dextrocardia
• Trisomy 18 - VSD
 Signs & Symptoms of Infective
Endocarditis
• H/o CHD or any procedures • Elevated temperature
• Fever • Tachycardia
• Chills • Embolic Phenomena – Roth spot,
• Chest & abdominal pain osler nodes, petechiae, splinter nail
• Dyspnea bed hemorrages
• • Janeway lesions
Night sweats
• • New or changing murmurs
Weight loss
• • Splenomegaly
CNS Manifestations
• Arthritis
• Heart failure
• Clubbing
• Metastatic infection
 Signs & Symptoms of PHT
• History of Congenital Heart disease
• Breathlessness, fatigue, syncope on exertion
• Chest pain
• Hemoptysis
 Signs & Symptoms of failure
• Feeding difficulty
• Hepatomegaly
• Edema on dependent parts
• Failure to thrive
• Feeding difficulty
• Forehead sweating
• Breathlessness
 Time of Onset of CCF in Congenital
lesions
Age Lesion
Birth to 72 hrs Pulmonary, mitral and aortic atresias or critical stenosis

4 days to 1 week Hypoplastic left and right heart syndromes, transposition and
malposition of great arteries with poor mixing

1 to 4 weeks Transposition and malposition complexes, endocardial

fibroelastosis, coarctation of aorta
1 – 2 months Transposition and malposition complexes, endocardial cushion
defects, VSD, PDA, TAPVC, ALCAPA
2 to 6 months Transposition and malposition complexes, VSD, PDA, TAPVC,
Aortic stenosis, Coarctation of Aorta
 Presence of CCF Excludes Tetralogy Physiology
except when complicated by
• Anemia
• Infective Endocarditis
• Valvular Regurgitation
• Surgically created or naturally occurring large left to
right shunts
• Systemic hypertension
• Unrelated or coincidental myocardial disease
Systemic Examination –
Auscultation
 Second Heart Sound S2
S2

A2 P2
SH, AR Accentuated PAH

Calc.AV, Aortic Atresia Diminished PS, PA

AS, PDA, AR, LVF, LBBB Delayed PS, ASD, TAPVC, RBBB

VSD, MR Early
 Spliting of Second Heart Sound
Spliting Expiration Inspiration

Normal

Wide & Variable

MR, VSD, PS

Wide & Fixed

ASD, TAPVC, RBBB

Paradoxical
AS, PDA, AR

Single Second Sound

TOF
 When to call a Murmur as Innocent or
Functional?
• Blood Pressure is Normal
• No Cardiomegaly
• No Cyanosis
• Second Sound is Normal
• X-ray Chest is Normal
• Electrocardiogram is Normal
 Acyanotic Patients with continuous
murmur
• Patent Ductus arteriosus
• Sinus of valsalva fistula to right atrium or ventricle
• Coronary arteriovenous fistula
• Systemic arteriovenous fistula
• Coarctation of the aorta
• Peripheral pulmonic stenosis
• Anomalous left coronary artery from pulmonary artery
(ALCAPA)
 Continuous Murmur in Cyanotic
Patients
• Bronchial collaterals in anomalies of Fallot’s
physiology
• Patent ductus arteriosus in patients with Fallot’s
physiology
• Total anomalous pulmonary venous connection
• Pulmonary arteriovenous fistula
• Surgically created shunts
Approach for Congenital Heart
Disease
Approach To Congenital Heart Diseases
• When to suspect heart disease in children?
• Symptoms of heart disease in children
• How to decide whether congenital or acquired?
• Age of onset of symptoms and time of diagnosis
• Markers of congenital heart disease
• History of acquired heart disease
Once Congenital Heart disease is
decided….

Apply NADAS’ Criteria

 NADAS’ Criteria
MAJOR MINOR
• Systolic murmur with thrill • Systolic murmur without
• Any diastolic murmur thrill
• Cyanosis • Abnormal P2
• Congestive cardiac failure • Abnormal BP
• Abnormal CXR
• Abnormal ECG

1 Major or 2 Minor Criteria indicates Presence of

Congenital Heart Disease
 Approach to Heart disease
Patient
Apply NADAS’ Criteria

Heart Disease Present Heart Disease Absent

Re-evaluate after
Acyanotic CHD Cyanotic CHD Six months

L  R Shunts
Obstructive
Lesions
Regurgitant
lesions
Acyanotic Congenital Heart
Disease
 Acyanotic CHD
Classification
Left to Right Shunts Obstructive Lesions Regurgitant lesions

Frequent resp. infections - Forcible heaving precordium Generally

Precordial buldge - Systolic thrill uncommon &
asymptomatic
Hyperkinetic precordium - No cardiomegaly
Tendency for sweating & CCF - Delayed corresponding
component second heart sound Eg. MR, AR, TR, PR
Shunt & flow murmurs
Plethoric lung fields on CXR - Ejection systolic murmur

Eg. ASD, VSD, PDA, AP -Ventricular hypertrophy on ECG

Window Eg. PS, AS, COA
 Acyanotic CHD : L → R Shunts

Left parasternal impulse Left ventricular type apial Wide pulse pressure
impulse
Wide, fixed split S2 LV type impulse
Systolic thrill
Pulmonary ejection systolic Systolic or continuous
murmur Pansystolic murmur thrill
Tricupid diastolic flow Mitral diastolic flow Continuous murmur
murmur murmur
Mitral diastolic flow
rsR‘ in V1 in ECG LV dominance in ECG murmur

Atrial Septal Defect Ventricular Septal Patent Ductus

Defect Arterious
Atrial Septal Defect – OS type
Ventricular Septal Defect - PM
Gerbode Defect
PDA
COA
 Acyanotic CHD – Obstructive Lesions

Right sided Left sided

Left parasternal heave Absent or weak, delayed

Narrow pulse pressure femorals compared to radials
Systolic thrill
Systolic thrill Arm blood pressure high
Ejection systolic
murmur in upper left Ejection systolic Prominent carotids, palpable
sternal border murmur radiating to aorta in suprasternal notch
neck Palpable collaterals
Wide split second
sound, delayed, well Delayed A2 Ejection murmur in inter-
heard P2 scapular region

Aortic
Pulmonary
Stenosis Coarcation of Aorta
Stenosis
Pulmonary Stenosis
Aortic Stenosis
Cyanotic Congenital Heart
Disease
 Cyanotic Congenital Heart Disease

Cyanosis, Clubbing, Polycythemia

With PS Without PS

No VSD With VSD Increased PA Normal PA

pressure pressure

Increased Pulm Decreased Pulm Pulm Venous

blood flow blood flow Obstruction
 Cyanotic Congenital Heart Disease
Cyanosis, Clubbing, Polycythemia

Pulmonary stenosis with right to left shunts at atrial level

Eg. Critical PS, Ebstein’s anomaly
Pulmonary stenosis with VSD – Fallot’s Physiology
Eg. TOF, DORV, Single ventricle, ECD, Hypoplastic RV
Increased blood flow with PAH – TGA Physiology
Eg. TGA, TAPVC, Tricuspid Atersia
Decreased pulmonary blood flow with PAH
Eg. Hypoplastic LV, Eisenmenger syndrome
Normal Pulmonary Artery pressure
Eg. TAPVC, Single Atrium, Pulmonary AV fistula.
CCHD - Pulmonary stenosis with right to
left shunts at atrial level
Prominent a waves – JVP Quiet precordium
Parasternal heave Heart size increased
Cardiomegaly may be present Multiple sounds ± gallop rhythm
Systolic thrill ± Scratchy systolic and diastolic
murmur
Cyanosis may be mild
Characteristic ECG
Inter-costal retractions present
RVH with late transition in ECG

Critical Pulmonary Ebstein’s Disease

Stenosis
Pulmonary Atresia
Ebstein’s Anomaly
Ebstein’s Anomaly - Echo
CCHD - Pulmonary stenosis with VSD –
Fallot’s Physiology
No Cardiomegaly, Mild left parasternal impulse, thrill
uncommon, S2 single, ejection murmur ends before S2, MPA
Absent, Ischemic lungs
ECG
RAD, RVH: TOF, DORV, Single Ventricle
RAD, LVH: Single ventricle, Hypoplastic right ventricle
LAD, RVH: Single ventricle, ECD with PS
LAD, LVH: Tricuspid atersia, Single Ventricle
Tetralogy of Fallot
 Differential diagnosis of Fallot’s
Physiology
• Fallot’s Tetralogy
• Transposition of great arteries
• Tricuspid atresia
• Single ventricle
• Double outlet right ventricle
• Corrected transposition of great arteries
• Atrioventricular canal defect
• Malpositions
CCHD - Increased blood flow with PAH –
TGA Physiology
Neonate or infant, Failure to thrive,
Congestive failure
Cardiomegaly
Radioloically – Cardiomegaly with
pulmonary plethora

d-TGA, DORV, Tricuspid Atersia, Single

Ventricle, TAPVC, Misc. Malpositions
 CCHD with Increased PBF
• Complete transposition of great vessels
• Double outlet right ventricle without pulmonary stenosis
• Tricuspid atresia with large VSD
• Persistent truncus arteriosus
• Single ventricle without pulmonary stenosis
• Total anomalous pulmonary venous connection
• Malpositions without obstruction to pulmonary blood flow
 Features of Eisenmenger Physiology
• History of frequent chest infection in infancy
• Cyanosis present from birth or appears late
• JVP – Prominent ‘a’ waves
• No cardiomegaly or thrill
• No parasternal heave
• Constant ejection click of PHT
• Second sound palpable, pulmonary component is accentuated
• Systolic murmur in pulmonary area – insignificant of absent
• Pulmonary and/or tricuspid regurgitation murmurs may be present
 Features of PAH due to Pulmonary
Venous Hypertension
• Mostly die with in 2 – 3 weeks if not treated
• Severe cyanosis from birth
• Congestive cardiac failure
• Absence of cardiac enlargement
• S3 Gallop
• Absence of murmurs
• CXR – Normal heart with Severe pulmonary venous
hypertension which results in ground glass appearance of
lungs
 CCHD - Decreased pulmonary blood
flow with PAH
Pulmonary Venous Hypertension

Present Absent
Relatively older patient
Hypoplastic left
heart, TAPVC with Features of PAH – Loud P2, RVH
Obstruction Classical radiological features of PAH, Main
pulmonary artery segment prominent if great
artery relation normal

Eisenmenger syndrome: ASD, VSD, PDA & Anomalies

of TGA physiology
 CCHD – Normal Pulmonary Artery
pressure
Cardiomegaly

Present Absent
Faint continous murmur on
Features of L →R Shunt
the chest wall
at Atrial Level

Superior vena
Pulmonary Arteriovenous cava to left atrial
TAPVC, Single fistula shunt
Atrium
AV Canal Defect
Tricuspid Atersia
 References
• Nelson Textbook of Pediatrics – 17th edition
• Pediatric cardiology – Myung K.Park – 4th edition
• Current pediatric diagnosis & treatment – 17th edition
• Perloff The Clinical Recognition of Congenital Heart
disease – 4th & 5th edition
• Feigenbaum’s Echocardiography – 6th edition
• A primer of ECG – K.P.Misra
Thank you