Surgical treatment of Marfan patients with aneurysms and dissection of the proximal aorta.

BACKGROUND: The authors retrospectively analyzed early and late results of surgical treatment for 79 Marfan patients with aneurysms and dissection of the proximal aorta.

METHODS: From September 1979 to February 1996, 79 patients with Marfan syndrome underwent aortic root replacement using composite grafts (n=68, Bentall-technique 63, button-technique 5), and ascending aortic replacement with a valve-sparing procedure (n=11). There were 12 patients (15.2%) who simultaneously received partial or total arch replacement. 55 patients (69.6%) were male, and 24 female (30.4%). The average age was 33.8 years. Forty-one patient (51.9%) had non-dissecting aneurysms while the remaining 38 patients suffered from either acute (24.0%) or chronic aortic dissection (24.0%). The aortic valve was involved in 97.5% of all cases.

RESULTS: The total early mortality (< or =30 days) was 3.8%, 10.5% for acute aortic dissection and 2.4% for non-dissecting aneurysms. There were no early postoperative deaths in patients after valve-sparing operation and in those with chronic aortic dissection. The follow-up rate was 98.7%. During a mean follow-up of 68+/-25 months 10 patients (13.3%) died and cardiac complications were a common cause of the late deaths. There was no late mortality in the valve-sparing operations during a mean follow-up period of 8+/-6 months, however, 1 required valve replacement. 19 (25.3%) of the 75 patients surviving late have undergone 25 secondary operations on the cardiovascular system. Reoperations at aortic sites distant from the original were much more frequent after primary repair for acute and chronic dissection when compared to non-dissecting aneurysms (p<0.005). Actuarial survival rate of all patients with composite graft replacement including early deaths was 91.2% at 1 year, 84.4% at 5 years and 75.2% at 10 years.

CONCLUSIONS: Composite graft insertion has become the gold standard for treating Marfan-patients with non-dissecting and dissecting aneurysms of the aortic root. Our early experience in 11 patients with valve-sparing procedures indicated that this,variant may be the better choice in selected patients.