Succesfull management of a giant pulmonary artery aneurysm associated with severe congenital pulmonary valve stenosis in an adult patient

Pulmonary artery aneurysms are rare entities usually associated with congenital anomalies that increase blood flow/pressure in the pulmonary circulation, like left to right shunts and/or severe pulmonary valve pathologies. Clear re-commendations about their management are lacking. We report on a case of severe congenital pulmonary valve stenosis associated with a giant pulmonary artery aneurysm, secondary right ventricular outflow tract obstruction and right to left atrial shunt. We discuss the diagnostic pathway, therapeutic approach and patient evolution. The case report proves that a complete imaging of the heart and blood vessels is mandatory with severe pulmonary valve stenosis, in order to better describe its repercussions on the right ventricle and pulmonary circulation. They might be under detected by simple tran-sthoracic echocardiography but their presence has important therapeutic implications.