Interrupted aortic arch
| Interrupted aortic arch | |
|---|---|
| Other names | IAA |
| Specialty | Cardiology, Cardiothoracic surgery |
| Symptoms | Cyanosis, circulatory collapse, differential blood pressure between the upper and lower extremities |
| Complications | Heart failure, shock, multi‑organ failure |
| Usual onset | Neonatal |
| Causes | Congenital developmental defect; frequently associated with DiGeorge syndrome (22q11.2 deletion syndrome) |
| Diagnostic method | Echocardiography, Computed tomography, Magnetic resonance imaging |
| Differential diagnosis | Coarctation of the aorta |
| Treatment | Surgical repair; administration of prostaglandin E1 to maintain ductal patency in the neonatal period |
| Prognosis | Poor without intervention; early surgical repair significantly improves outcomes |
| Frequency | 3 per million live births |
Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch (especially Type B) is often associated with DiGeorge syndrome.