A successful surgical case of aortopulmonary septal defect was reported. The patient was a 12-month-old girl, who had aortopulmonary septal defect, ventricular septal defect, long narrow right pulmonary artery and left pulmonary hypertension. Aortography and echocardiography revealed a communication between the ascending aorta and the main pulmonary artery. Ventricular septal defect with bidirectional shunt was detected by right ventriculography. Operation was successfully performed under the extracorporeal circulation. Aortopulmonary septal defect was 14mm by 18mm in size. This defect was closed through aortomy using a Dacron patch and the ventricular septal defect was directly closed through right ventriculotomy. The patient recovered uneventfully. Postoperative cardiac catheterization was performed two months after operation. Right ventriculography revealed the long narrow right pulmonay artery and moderate tricuspid regurgitation. Right ventricular peak systolic pressure decreased to 56mmHg from preoperative value of 95mmHg. She was discharged two months after operation. Ventricular septal defect was detected in 5 of 56 patients undergoing surgical treatment of aortopulmonary septal defect in Japan. The defect with ventricular septal defect and long narrow right pulmonary artery was, to our knowledge, not found in Japanese literature ex-cept for our case.
