SIMULTANEOUS AORTIC ROOT REPLACEMENT AND REPAIR OF SUBCLAVIAN ARTERY ANEURYSM IN PATIENT WITH MARFAN SYNDROME

Abstract

Patients with Marfan syndrome frequently develop aneurysms or dissections involving multiple segments of the aorta and major arteries and occasionally require staged replacement of different portions of the aorta. We are presenting a Marfan patient with severe sternal and vertebral deformity and simultaneous presence of a dissected aortic root aneurysm and a saccular aneurysm of the right subclavian artery. The aortic root was totally replaced including the aortic valve and the ascending aorta, and the right subclavian artery aneurysm was bypassed by an aorta-axillary graft with exclusion of the aneurysm. Post-op course was complicated by repeated attacks of ventricular fibrillation based on long Q-T intervals, which was successfully managed by mexiletine. The case is reported and general management of Marfan patients discussed