Unilateral Pulmonary Artery Agenesis in an Elderly Female.

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly due to a malformation of the sixth aortic arch of the affected side during embryogenesis. It can occur in isolation or may be associated with other congenital cardiovascular malformations. The diagnosis is usually set at adolescence, however it can remain asymptomatic and late diagnosis is possible. Recurrent pulmonary infections, decreased exercise tolerance, and shortness of breath on exertion are the most common symptoms.