Staged surgical management of pulmonary atresia with diminutive pulmonary arteries.

A particular problem in treatment is presented by patients who have atresia of the pulmonary valve and ventricular septal defect but whose pulmonary arterial tree is extremely diminutive. The pulmonary blood flow results from naturally occurring large bronchial collateral arteries. In the experience reported, satisfactory palliation was provided for all 3 patients in whom right ventricle--to--pulmonary artery continuity was constructed by placing a pericardial patch graft during open-heart operation. The ventricular septal defect was left unrepaired. Early evidence supports the hypothesis that, besides providing palliation of cyanosiis, the procedure may allow progressive enlargment of the hypoplastic pulmonary arteries. This offers hope that later second-stage completion of total repair, involving closure of the ventricular septal defect and ligation of any remaining large bronchial collateral arteries, may become feasible.