Objective: Evaluation of surgical treatment of interrupted aortic arch (IAA) by direct anastomosis. Methods: A consecutive series of 17 infants with IAA (type A in eight patients, type B in nine) were operated upon. The mean age at arch repair was 1.0 month (range 0.2-7.7), mean weight was 3.7 kg (range 2.2-6.2). All arch repairs were done by direct anastomosis. This included a persistent arterial duct in one and a subdavian turnup in another case. The aortic reconstruction included reimplantation of a lusoric artery in three patients, patch enlargement of the ascending aorta in three and of the complete arch in one patient. The arch repair was done through a lateral thoracotomy in three patients. In 14 patients the aortic repair was part of a single-stage approach through a median sternotomy using cardiopulmonary bypass and circulatory arrest. Results: There was no operative mortality. One patient (single-stage approach) died 2 days after operation due to respiratory problems caused by tracheobronchomalacy. One patient (lateral approach) died suddenly 3 months after aortic repair and banding. Median follow up was 4.8 years (range 0.1-12.9). In five patients restenosis of the aortic arch developed, all within 1.5 years after repair. This was not correlated with the type of interruption, weight at operation, age at operation or the surgical approach. The actuarial freedom from restenosis was 61% at 5 years with a 70% confidence limit (CL 70% ) of 46-75. All restenoses were balloon dilated, but two needed redo surgery, which was done by the median approach. In three patients discrete subaortic stenosis developed. This was not correlated with the type of interruption, weight at operation, age at operation or the surgical approach. The actuarial freedom from subaortic stenosis was 68% at 5 years (CL 70% = 54-83). These stenoses were treated by enucleation, followed in one patient by a pulmonary autograft procedure for recurrent root stenosis after another year. At the end of follow up all patients were thriving well, lacked symptoms, were normotensive and had normal femoral artery pulsations. Conclusions: IAA can be treated well with primary anastomosis. Possible restenosis of the aortic arch can adequately be treated by percutaneous balloon dilatation or redo surgery if necessary. Arch repair by median single-stage approach has our preference.
