Aneurysm of the main pulmonary artery: long-term survival after aneurysmorrhaphy and closure of a ventricular septal defect.

Pulmonary artery aneurysm (PAA) usually carries an ominous prognosis due to the associated pulmonary hypertension. Six years ago a patient with a PAA secondary to cystic medial necrosis and pulmonary hypertension due to a ventricular septal defect (VSD) was treated by aneurysmorrhaphy and closure of the VSD. Although the patient had early postoperative cardiac failure, his cardiopulmonary status stabilized, and he has done well without recurrence of his aneurysm or cardiac failure. This report summarizes the patient's clinical course, operative treatment, and long-term follow-up.