Background. Increased pulmonary vascular resistance is common in congenital heart disease and is exacerbated by cardiopulmonary bypass (CPB). We investigated whether CPB is responsible for pulmonary endothelial dysfunction and contributes to postoperative pulmonary hypertension.

Background. Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses.Methods and Results. Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only;

Small right heart size is associated with RVDCC but is not associated with survival in PA-IVS. Patients without RVDCC have improved survival after RVD regardless of neonatal right heart size.

A female infant who had been misdiagnosed as having primary pulmonary hypertension of the newborn was found to have agenesis of the branch pulmonary arteries with collateral-dependent pulmonary circulation. The intracardiac anatomy was completely normal. Unifocalization of branch pulmonary artery was done, followed by stenting of the ductus arteriosus.