Results of aortic surgery in patients with Marfan syndrome.

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

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.