Introduction
Pulmonary artery aneurysm may be associated with congenital cardiac
anomalies over 50% of the time, including pulmonary artery
stenosis.1 There are few reports of isolated
congenital pulmonic valve stenosis with associated pulmonary artery
aneurysm, and no reports of open repair in a patient with Alpha-1
Antitrypsin deficiency.2, 3 We present a case of a
patient with Alpha-1 Antitrypsin deficiency who presented with
idiopathic congenital pulmonic valve stenosis, with findings of a
pulmonary artery aneurysm, and successful open operative repair.