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.