Extensive aortic surgery in Marfan syndrome: 16-year experience

The aim of this study was to evaluate the clinical outcome of surgical treatment in patients with Marfan syndrome. Between 1985 and November 2001, 33 patients with Marfan syndrome were operated for chronic aneurysm of the aortic root with involvement of the ascending aorta in 20 patients and type A dissection in 13 patients. The patients comprised 24 males and 9 females with a mean age of 31.9 +/- 9.7 years (range, 18 to 54 years). The mean diameter of the ascending aorta was 6.6 +/- 1.6 cm and that of the aortic root was 5.4 +/- 1.2 cm. Hemodynamic instability was observed in 11 patients. The aortic arch was replaced in 7 patients. There was no hospital mortality. Late mortality was 6%, involving 2 patients who had aortic valve replacement. Actuarial freedom from death was 92.3% +/- 7.4% at 12 years and from late aortic complications was 86.4% +/- 9.4% at 13 years. Aortic aneurysm was a significant univariate adverse factor for late aortic complications. Aortic surgery can be performed in Marfan patients with low morbidity and mortality. Aggressive surgical intervention does not impair surgical outcome while it decreases reoperation risk.