Pulmonary artery aneurysm (PAA) is a rare disorder that may be classified as congenital, acquired, or idiopathic, in the case of unclear etiology. When associated with severe idiopathic pulmonary arterial hypertension, such a case of PAA may present to the operating room as an indication for lung transplantation. In this article, we present such a case of a patient with a giant main and right PAA that underwent a double lung transplant. We describe the pathophysiology and natural course of this PAA and discuss the role of intraoperative transesophageal echocardiography in the management of patients with this rare diagnosis.

No abstract