Interrupted Aortic Arch Associated with Complex Congenital Heart Disease

Interrupted aortic arch (IAA) is a rare congenital malformation of the aortic arch, which occurs in 3 per million live births. This anomaly is defined as a loss of luminal continuity between the ascending and descending portions of the aorta which entails a very poor prognosis without surgical treatment. In most cases, IAA is associated with intracardiac malformations such as VSD, PDA, bicuspid aortic valve, left ventricular outflow tract obstruction or aortopulmonary window. We report a 16-year-old boy with IAA associated with double inlet left ventricle, aneurysmal dilatation of pulmonary artery, giant PDA and severe pulmonary hypertension. To our knowledge, there is no previous report of IAA associated with complex congenital heart disease such as that seen in our case (Iranian Heart Journal 2007; 8 (3): 60-63).