Pulmonary Atresia with Ventricular Septal Defect: Rare Presentation with Coronary-to-Pulmonary Artery Collaterals from Both Right and Left Coronaries

Abstract

Pulmonary atresia with ventricular septal defect and coronary-dependent pulmonary circulation arising from both major coronary arteries is rare. Dependence of pulmonary blood flow on the coronaries and the risk of early development of pulmonary vascular obstructive disease warrant early surgical repair in these patients. We report a case of a ten-month-old infant with pulmonary atresia with ventricular septal defect and coronary artery-to-main pulmonary artery connections who was successfully managed with ligation of the coronary fistulas and intracardiac repair.