Pulmonary atresia with ventricular septal defect in adult patients

Hemodynamic data and angiograms of 15 adult patients with pulmonary atresia and ventricular septal defect were reviewed to assess the pulmonary circulation and other associated features. The most common variety of pulmonary atresia was that of pulmonary valve, main pulmonary artery, and the confluence of pulmonary arteries (6 cases, 40%). The collateral vessels to the lungs were well developed in all cases; selective injections into the collateral vessels were of great value in their delineation. The left ventricle was well developed in 11 cases (73.3%). Congestive heart failure was seen in 8 (53.3%), tricuspid regurgitation in 10 (66.7%), and aortic regurgitation in 7 (46.7%) cases. The long survival in these patients was related to the favorable anatomy of central pulmonary arteries (12 cases, 80%) and adequate pulmonary collateral circulation.