A neonate in congestive heart failure has echocardiographic evidence of a single truncal vessel from which the pulmonary arteries arise, a VSD and truncal valvar stenosis. The following is/are true:

a. Natural history of this anomaly allows only 20% one-year survival

d. Repair of the lesion requires an extracardiac conduit

The defect described is truncus arteriosus which carries an 80% one year mortality rate uncorrected. The truncal valve is most commonly tricuspid (65%) or quadricuspid (25%); least likely bicuspid (9%). The large left-to-right shunt makes these patients particularly likely to develop pulmonary vascular obstruction (Eisenmenger’s syndrome). Operative repair requires detachment of the pulmonary arteries which are reconnected to the right ventricle by an extracardiac conduit, and the optimal timing for repair is within the first 6 months of life.