Ann IM 2001;135:812; Nejm 2000;342:256

Cause:Congenital malformation

Pathophys:

Causes a left-to-right shunt which in end-stage disease reverses to a right-to-left shunt (Eisenmenger's syndrome) with clubbing and cyanosis

Size of defect is crucial to sx, location unimportant. At birth, high hematocrit keeps PA pressure up and thus low shunt flow and minimal murmur; with dropping crit postpartum these appear (Nejm 1982;306:502)

20% of all CHD; associated with triad of 3rd-degree heart block, ventricular septal defects, and corrected transposition

Sx:Usually asx; can develop dyspnea, fatigue, and pulmonary infections

Si:Loud harsh pansystolic murmur at lower left sternal border, widely transmitted over precordium. Apical diastolic rumble if large shunt; loud P₂ if pulmonary hypertension

Spontaneous closure in 1/3 in childhood; life expectancy >65 yr if shunt <2:1

SBE (5-30% lifetime risk), CHF, pulmonary hypertension; with Eisenmenger's, 2/3 die in pregnancy (Nejm 1981;304:1215)

Lab:

Noninv:EKG normal unless huge left-to-right shunt causing LVH; RVH suggests fixed pulmonary hypertension

Echo w Doppler

Xray:Chest usually normal; with larger defects causing LVH and prominent pulmonary vasculature and a large left atrium. Small aortic knob

Rx:

SBE prophylaxis

Surgical indications: left-to-right shunt >2:1 or CHF; PA banding in infants; optimal age for final repair is 5 yr; contraindicated if right-to-left shunt. Operative mortality: 1-5%, increases to 15% if pulmonary HT (>70% of systemic pressures); banding mortality 25%. Surgical cmplc: complete heart block (rare), aortic insufficiency (rare)