Results of aortic surgery in patients with Marfan syndrome

We reviewed the outcome of aortic operations in patients with Marfan syndrome to evaluate the immediate and long-term results of surgical treatment.

Between January 1985 and June 2002, 22 patients with Marfan syndrome underwent surgical treatment for aortic diseases at our hospital. Eight patients had Stanford type A aortic dissection and ten patients had aortic root aneurysm with aortic regurgitation. We performed aortic root replacement in 21 patients. Subsequent aortic operations were done in 11 patients, 6 of whom required a third operation. Five patients underwent repeat surgery of the previously operated aortic segment. A collective a total of 42 aortic operations were performed in the 22 patients. Seven patients underwent total aortic replacement.

The in-hospital mortality rate was 4.5%, and there were three late deaths: two caused by rupture of the dissected aorta, 17 months and 24 months after the last surgical intervention, respectively, and one caused by heart failure 27 months after a total aortic replacement. The overall actuarial survival was 90.2% at 5 years and 74.4% at 10 years.

Surgical treatment of patients with Marfan syndrome can be accomplished with low mortality; however, new aortic lesions should be promptly explored, and appropriately timed surgical treatment can substantially improve the prognosis of patients with Marfan syndrome.