Aortopulmonary septal defect coexisting with ventricular septal defect and pulmonary atresia.
Three patients are described in whom an aortopulmonary septal defect (aortopulmonary window) coexisted with a ventricular septal defect and pulmonary atresia. One patient had mild and another, moderate aortic regurgitation. In addition, one patient had a sinus of Valsalva aortic aneurysm, while another had a single coronary artery arising from the pulmonary trunk. One patient underwent surgical correction in infancy; the other two, in early adult life. In all three patients, surgical correction involved closure of the aortopulmonary window, closure of the ventricular septal defect, and placement of a valved conduit between the right ventricle and the distal pulmonary trunk. One patient died 3 weeks postoperatively due to secondary hemorrhage where the conduit had been sutured to the right ventricle. The other two patients are alive and well 3 1/2 years and 6 months after surgery, respectively. The presence of aortopulmonary window permits normal development of the pulmonary arteries in the presence of the coexisting pulmonary atresia; in the three patients described, the absence of pulmonary vascular disease made total surgical repair feasible for this combination of defects.
D000328:Adult; D001011:Aorta / Q000002:abnormalities*; D001014:Aortic Aneurysm / Q000150:complications / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000150:complications / Q000601:surgery*; D003331:Coronary Vessels / Q000601:surgery; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000473:pathology / Q000601:surgery*; D006352:Heart Ventricles / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000000981:diagnostic imaging / Q000601:surgery; D011859:Radiography; D012850:Sinus of Valsalva