Surgical Repair of Aortic Atresia in an Adult

Aortic atresia is a rare congenital vascular anomaly that occurs in adults. Differentiating it from interrupted aortic arch without open surgery is difficult. In some patients with type A interrupted aortic arches, aortic atresia may actually be caused by aortic coarctation. A 38-year-old man was diagnosed with aortic atresia, aortic regurgitation with a bicuspid valve, and aortic root enlargement. The surgery was performed in 2 stages. First stage: Descending aortic replacement with partial cardiopulmonary bypass without cardiac arrest. Second stage: Bentall procedure with mechanical valve replacement. We discuss the pathophysiology of interrupted aortic arch and aortic atresia in adults.