A complex case of congenital cardiac anomaly: pulmonary atresia and ventricular septal defect associated with major aortopulmonary collaterals

Abstract

Pulmonary atresia and ventricular septal defect associated with major aortopulmonary collaterals (MAPCAs) is a rare, complex, and heterogeneous congenital cardiac anomaly. The majority of untreated patients present with severe congestive heart failure and respiratory distress in the first decade of life. We describe a 15-year-old cyanotic boy, both of whose pulmonary arteries arise from the arcus aorta via patent ductus arteriosus. In addition to this anomaly, the patient has MAPCAs originating from the descending aorta that perfuse the right upper lobe of the lung, a persistent superior vena cava, an aberrant right subclavian artery, pulmonary atresia, and ventricular septal defect.