Correction of pulmonary atresia with ventricular septal defect in the absence of the pulmonary trunk and the central pulmonary arteries (so-called truncus type IV).

A child with pulmonary atresia and ventricular septal defect and no extraparenchymal pulmonary arteries had all the bronchopulmonary arterial segments connected to naturally occurring systemic-pulmonary collaterals. A three-staged surgical correction was performed. At the first and second stages, the arteries of each hilus were interconnected with synthetic arteries. At the third stage, the ventricular septal defect was closed, and continuity between the right ventricle and the bilateral pulmonary circulation was established with a valved conduit giving rise to a side arm. Postoperative evolution was good, with acceptable postrepair per right ventricular--left ventricular pressure ratio.