Large pulmonary artery aneurysm associated with Marfan syndrome

Abstract

Pulmonary artery aneurysms are rare and published data on the subject are limited. The first case of Marfan syndrome associated with a large pulmonary artery aneurysm complicated by pulmonary artery thrombi and pulmonary hypertension is described. Serial echocardiograms showed progressive dilation of the aneurysm, which reached a massive size of 90 mm. Cardiac catheterization, pulmonary angiography and computed tomography angiography were used in the preoperative evaluation. The patient died suddenly awaiting a decision for surgical intervention, thereby underscoring the need for aggressive management.