Interrupted Aortic Arch

Interrupted aortic archInterrupted Aortic Arch is a rare congenital defect whereby the aortic arch is discontinuous resulting inDuct dependant Circulation a duct dependant systemic circulation. Classification is based the anatomical location of interruption. Type B is most common, whereby there is an interruption between the left common carotid artery and the left subclavian artery. Primary surgical repair is required in the first few days after diagnosis unless contraindications to cardiopulmonary bypassCardiopulmonary Bypass exist. Almost all cases will have a significant intracardiac shunt or AP window. Common associated anomalies include a posterior malaligned ventricular septal defectVentricular septal defect (VSD) which can be associated with left ventricular outflow tract obstructionLeft ventricular outflow tract obstruction and, rarely, hypoplastic left ventricleVentricles. Up to 50% of patients with interrupted aortic archInterrupted Aortic Arch have DiGeorge syndromeTruncus-interruption. Surgical outcomes have improved in contemporaryAorto-pulmonary window cohorts.