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Objective To discuss one-stage surgical repair of interrupted aortic arch ( IAA) in neonates and young in-fants, summarize follow-up results and subsequent treatments.Methods From September 2010 to December 2014, a series of 38 consecutive neonates and young infants ( M/F =26/12 ) with IAA were admitted in our surgical group to receive surgical treatment, with a median age of 11 days(1 day to 5 months) and a median body weight of 4.1 kg(1.8 to 5.8 kg).IAA was type A in 24 cases, type B in 13 cases and type C in 1 case.Associated cadiovascular anomalies were common except 2 cases, including atrial septal defect(n=23), ventricular septal defect(n=36), left ventricular outlet tract obstruction(LVOTO)(n=7), anomalous left pulmonary artery from aorta(n=1), truncus arteriosus(n=1).There were 5 cases of airway stenosis in 28 cases of CT angiography and 3D airway reconstruction.All the 38 cases were admitted with differential cyanosis.36 cases were recieved one-stage surgical repair except 2 cases died before operation.35 cases of IAA associated with intracardiac anom-alies were repaired through a median sernotomy.Cardiopulmonary bypass(CPB) was performed with two canulations in ascend-ing aorta and main pulmonary artery.Selective cerebral perfusion(n=21) or circulatory arrest(n =14) was carried out with body temperature of 18℃-23℃.After the patent ductus arteriosus tissue was completely resected , a continuous end-to-end or end-to-side suturing with 7/0(8/0) prolene was performed.In 1 type B cases with expected excessive anastomotic tension, the left subclavian artery was cut off to release the aortic arch .The associated anomalies were repaired during the cooling or re-warming period.Chest close was delayed to carry out no more than 24-72 h postoperatively in 16 cases.The other one type A IAA case without intracardiac anomaly was repaired through a standard left posterior lateral incision .Patients were strictly fol-lowed up with a standard protocol.Complications such as aortic arch restenosis, new LVOTO, new airway stenosis, and so on, were specialy focused on during the follow-up period.If needed, reoperation or transcatheter intervention was applied to treat the complications.Results Two cases died before operation and another 2 died in the early period postoperatively because of pulmonary hypertension crisis and severe tracheal stenosis, respectively.Sudden death was in 1 case during the follow-up peri-od.Median CPB time was 138 min(105-208 min) and median selective cerebral perfusion or circulatory arrest time was 24 min(16-35 min) .Mechanical ventilation time was 2-25 days.Blood pressure difference between the upper and lower limbs was less than 20 mmHg in all the patients before discharge.29 cases(85.29%) was followed up for 28 months(9 -60 months).3 cases were received reoperation in 5 cases of newly detected LVOTO.One case received reoperation in the 7 preop-erative LVOTO cases because of newly detected LVOTO(blood pressure gradient more than 40 mmHg).Two cases with aortic arch restenosis received percutaneous transcatheter ballon dilation .One case with new left main bronchus stenosis after opera-tion was strictly followed up.Conclusion IAA is a rare and severe congenital heart disease.Positive surgical repair should be performed after definite diagnosis being made .Although satisfactory results can be obtained with one-stage primary operation , IAA is a kind of progressive chronic disease.New postoperative LVOTO should be focused on becasue it will need reoperation or even the third operation .