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Interrupted aortic arch (IAA) is a rare form of critical neonatal heart disease in which there is lack of continuity between the ascending aorta and the descending thoracic aorta. In the absence of prenatal diagnosis, patients with IAA present in shock when the patent ductus arteriosus closes. Diagnosis can generally be made by echocardiography, and initiation of prostaglandin E1 infusion allows for adequate lower body perfusion prior to surgical repair. Full neonatal repair can be achieved with good outcomes in most cases. However, there is often underdevelopment of the left ventricular outflow tract and risk for later surgical re-intervention. Many patients with IAA, particularly those with type B, have DiGeorge syndrome, which has important development implications.