Congenital heart disease

Cyanotic (5 T's) vs acyanotic shunts, when to suspect, what to image, when to fix.

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◆Acyanotic — left-to-right shunts

•VSD (most common CHD): holosystolic harsh murmur at LLSB; small ones close spontaneously; large → CHF in infancy → surgical closure
•ASD: fixed split S2, soft systolic ejection murmur at ULSB from increased pulmonic flow; often asymptomatic until adulthood; close if symptomatic or large
•PDA: continuous 'machinery' murmur at LUSB; preterm infants → close with indomethacin; term/older → surgical or catheter closure
•AV canal defect: associated with Down syndrome; CHF early; surgical repair
•All untreated L→R shunts can develop Eisenmenger syndrome → cyanosis from reversed shunt

•Tetralogy of Fallot (most common cyanotic past infancy): pulmonary stenosis, VSD, overriding aorta, RVH; 'tet spells' → squat or knee-to-chest; surgical repair
•Transposition of great arteries: severe cyanosis at BIRTH; need PGE1 + balloon septostomy → arterial switch (Jatene)
•Tricuspid atresia: cyanosis + LVH + decreased pulmonary markings; staged Fontan procedure
•Truncus arteriosus: single common trunk; CHF + cyanosis; early surgical repair
•TAPVR (total anomalous pulmonary venous return): 'snowman' on CXR if supracardiac type
•Hypoplastic left heart syndrome: poor systemic perfusion, gray baby; PGE1 + Norwood/Sano + Fontan staged

•Coarctation of aorta: HTN in upper extremities, weak femoral pulses, BP discrepancy; associated with Turner syndrome and bicuspid aortic valve; rib notching on CXR (older kids); surgical or catheter repair
•Aortic stenosis (congenital, bicuspid): late presentation with syncope, angina; rarely critical in newborn (ducts-dependent)
•Ebstein anomaly: lithium exposure; tricuspid valve displaced into RV; arrhythmias
•Critical pulmonary stenosis: ductal-dependent in newborn

•Cyanotic newborn: hyperoxia test (PaO2 fails to rise above 100 on 100% O2 → cardiac cause)
•Echo is the diagnostic test of choice for almost all CHD
•Ductal-dependent lesions (HLHS, transposition, severe coarctation, pulmonary atresia) → KEEP DUCT OPEN with PGE1
•Eisenmenger physiology: established pulmonary HTN reverses shunt → cyanosis → contraindication to repair
•All CHD patients need endocarditis prophylaxis ONLY if: unrepaired cyanotic, prosthetic material <6 mo post-op, residual defect adjacent to prosthetic

Finding	Likely lesion
Holosystolic murmur LLSB in infant	VSD
Fixed split S2	ASD
Continuous machinery murmur	PDA
Cyanosis + boot-shaped heart, 'tet spells'	Tetralogy of Fallot
Severe cyanosis at birth, 'egg on string'	Transposition (TGA)
Differential BP arms vs legs, rib notching	Coarctation of aorta
AV canal in Down syndrome	AVSD
Continuous machinery + maternal rubella	PDA from congenital rubella

◆Cyanotic CHD = the 5 T's: TOF, TGA, Tricuspid atresia, Truncus, TAPVR (+ HLHS)
◆Ductal-dependent lesions in newborn → PGE1 (prevents closure of PDA, maintains shunt)
◆Tet spells (cyanotic spell in TOF) → knee-to-chest (increases SVR, decreases R→L shunt)
◆Down syndrome + heart defect → most often AV canal (endocardial cushion defect)
◆Turner syndrome + CHD → coarctation or bicuspid aortic valve
◆Maternal lithium → Ebstein anomaly
◆Eisenmenger physiology is a CONTRAINDICATION to closure (need lung-heart transplant)

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