Severe ascending aortic stenosis after one-stage repair of aortopulmonary window and interrupted aortic arch

A 33-day-old girl was transferred to our hospital because of severe cyanosis and tachypnea. The diagnosis of aortopulmonary window and interrupted aortic arch was established by echocardiogram and cineangiocardiogram. Reconstruction of aortic arch by extended direct anastomosis and simple patch closure of aortopulmonary window were performed by means of profound hypothermia and circulatory arrest on 36 days of age. Her postoperative clinical course was uneventful, but at 2 months after operation, stenosis of the ascending aorta was noticed by echocardiographic examination. As her physical growth had been satisfactory, she was managed under close observation at outpatient clinic. At seven months after operations, she fell into progressive left heart failure, and emergency operation was carried out for the relief of stenosis of the ascending aorta, but she could not come off bypass. A stenosis of the ascending aorta is a fatal complication that may occur after one-stage repair of interrupted aortic arch with aortopulmonary window consisting of extended direct anastomosis and simple patch closure. We now consider that division of ascending aorta and pulmonary artery and repair of both of the defect is indispensable for the correction of aortopulmonary window, especially in neonate and small infant with this lesion associated with interrupted aortic arch.