One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch

Absence of the aortopulmonary septum, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus is a rarely reported association. A 3-year-old boy underwent successful one-stage repair of this constellation of anomalies. A Dacron baffle was used both to close the huge aortopulmonary window and to direct blood to the right pulmonary artery. Type A interrupted aortic arch was repaired by direct anastomosis. Postoperatively, pulmonary artery pressure was less than half systemic pressure.