Giant-Size Main Pulmonary Artery Aneurysm in an Adult Patient with Ebstein Anomaly and Dextrocardia

Abstract

Main pulmonary artery aneurysms are rare, mostly asymptomatic and discovered accidentally. The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension, Behcet’s disease, connective tissue disorders, congenital heart disease, vasculitis, syphilis, tuberculosis and endocarditis. There are some indices that dextrocardia is associated with anomalies of the pulmonary arteries and pulmonary valve. A rare occurrence of main pulmonary artery aneurysms results in a lack of recommendations, so the remaining challenges are whether main pulmonary artery aneurysms should be treated, how, and when. The decision on surgical intervention or conservative treatment of the main pulmonary artery aneurysms depends on its size, etiology and accompanying diseases and includes a multidisciplinary heart team. Our case of the main pulmonary artery aneurysm and pulmonary valve abnormality associated with Ebstein anomaly and dextrocardia in a 67-year-old male patient brings causality considerations and treatment options in such a unique condition.