Aorta–to–Pulmonary Vein Fistula in an Asymptomatic 25-Year-Old Man

Abstract

A 25-year-old man with no significant medical history was found to have a continuous murmur along the left sternal border on routine physical examination performed in preparation for running a marathon. Echocardiography showed a dilated left ventricle with normal function and continuous high-velocity flow in an aberrant vessel in close vicinity to the abdominal aorta (Movie I in the online-only Data Supplement). Chest x-ray (posterior-anterior and lateral projections, Figure 1) disclosed conglomeration of tubular densities in a scimitar pattern in the right lower lobe suggestive of anomalous pulmonary venous return of right lower lobe (scimitar syndrome; anomalous drainage of right lung pulmonary veins into the inferior vena cava). Cardiac magnetic resonance imaging (MRI) not only demonstrated normal drainage of all 4 pulmonary veins into the left atrium but also revealed an aberrant vessel (Movie II in the online-only Data Supplement) extending between the abdominal aorta and the right inferior pulmonary vein with an elevated systemic-to-pulmonary flow ratio of 1.8, consistent with a large left-to-left shunt. Cardiac computed tomography (CT) confirmed the presence of a large fistulous connection between the abdominal aorta and the right inferior …