Abstract:Objective To summarize the experiences of single stage repair of interrupted aortic arch （IAA） associated with cardiac anomalies. Methods From Jan. 2000 to Dec. 2005, 48 patients admited in hospital and 35 patients were operated, the mean age at operation was 1.1 years. The associated anomalies included 23 cases of ventricular septal defect, 2 cases of transposition of great arteries, 3 cases of aortopulmonary window with aortic origin of right pulmonary artery, 2 cases of truncus arteriosus, 2 cases of double outlet right ventricle, 2 cases of stenotic fifth arch and 1 case of aberrant origin of right subclavian artery with mild hypoplastic decending aorta. Among them, 34 patients underwent single stage repair and 1 kid underwent palliative correction. Results There were 4 surgical deaths. The sequelae included one diaphragm paralysis and one 3rd degree of atrioventricular block. Only 5 kids recurred mild stenosis of aortic arch anastomosis and 2 death occurred during 3 months to 4 years of follow-up. Conclusion Though early surgical mortality for primary single stage repair is now relatively low, if appropriate interventions has been accomplished during perioperative period, but outcomes of IAA remain of concern, especially in patients with associated lesions.