Congenital Aortic Arch Interruption and Hypoplasia

Abstract

Interrupted aortic arch (IAA) is a rare condition, most commonly associated with a ventricular septal defect. Without treatment it is lethal but the use of prostaglandin E1 infusion allows perfusion of the lower body via the duct and a degree of temporisation. Echocardiographic diagnosis is often sufficient but in selected cases computed tomography or angiography may be necessary. Depending on the interruption site and other anatomic features, the repair can be done via thoracotomy or sternotomy. A staged procedure with pulmonary banding or a hybrid strategy are also part of the armamentarium. Most groups will favour a complete repair via sternotomy as soon as the patient is stabilised. The arch is reconstructed with a direct anastomosis and patch augmentation is frequent. Cerebral perfusion techniques are increasingly prevalent. The early results have continued to improve but there is a significant decline in survival and functional status in the long-term, attesting to the chronicity of this condition.