Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8kg[mean weight, 4.0$pm$l.4kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1$pm$3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients[86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septurn was resected whenever necessary. There were seven early deaths[16.8% , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis,stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily.We conclude that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are resonable.