Abstract: Objective: To summarize the experience with one-stage repair of interrupted aortic arch（IAA） through midline sternotomy. Methods: Eight patients （male5,female3） with IAA were operated. The age of surgical operation was 1-11 years and body weight was 9-21kg, five patients were tape A IAA and 1 was type B. All patients had associated cardiac anomalies and severe pulmonary hypertension. Results: There was one early death as a result of severe low cardiac output syndrome combined with acute renal failure. The 6 survivors were followed up from 0.5-14 years,and no late death. One patient required a bypass with artificial graft between ascending aorta and descending aortic artery who had aortic homograft calcification and residual pressure gradient 12kPa after 12 years′ discharge. One had residual pressure gradient 5.3kPa.The other 4 patients had 1.3-3.3kPa, but their cardiac function was NYHA Ⅰ. Conclusion: The method of one-stage repair of IAA especially with direct end-end anastomosis through midline sternotomy can achieve good exposure, less trauma and excellent recuperation.