Interrupted Aortic Arch in an Adult

Interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment. To our knowledge, the world medical literature contains only 12 reports of isolated interrupted aortic arch diagnosed in adults. Nine of these patients underwent successful surgical repair, but 1 died during the early postoperative period. We describe a 10th successful surgical repair, which involved a 42-year-old woman who had an asymptomatic type B interrupted aortic arch (characterized by interruption between the left subclavian and left carotid arteries). We performed a single-stage extra-anatomic repair by placing a 16-mm extra-anatomic Dacron graft between the ascending and descending portions of the thoracic aorta and by interposing a 7-mm extra-anatomic Dacron graft between the 16-mm graft and the left subclavian artery. The patient recovered uneventfully and continued to do well 6 months later. (Tex Heart Inst J 2002;29:118–21)