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.