Interrupted Aortic Arch

Interrupted aortic arch (IAA) is a rare anatomic finding that accounts for approximately 1.5% of all congenital heart anomalies. The incidence of IAA is 3 per million live births and it is commonly associated with patent arterial duct, bicuspid aortic valve, and isolated ventricular septal defect (VSD). Prenatal diagnosis of congenital heart disease by ultrasound is becoming increasingly common. IAA is a difficult diagnosis to make in fetal screening based on a standard four-chamber view. Accurate anatomic diagnosis can currently be made using echocardiography alone. It is possible to complete the dissection, mobilization, and anastomosis with a circulatory arrest time of less than 25 minutes, which is unlikely to lead to any detectable neurologic consequences. Following biventricular repair of simple IAA/VSD, postoperative management should be routine. Failure to progress appropriately should stimulate an aggressive search for residual hemodynamic lesions.