Methods: Between February 1987 and October 2008, 62 patients with Marfan syndrome (45 males, 17 females; mean age 31.5±9.9 years; range 11 to 56 years) underwent surgery. A total of 90 surgical interventions were performed on these patients. Univariate and multivariate analyses of the risk factors were performed to evaluate the effect of these interventions on survival.

Results: Mean follow-up was 7.9±4.2 (range 0.2 to 17.4) years, giving a total follow-up of 471.2 patient/years. Early mortality was reported in two of the patients (3.2%). Survival rates at one, six and 15 years were 96.8±2.2%, 94.4±3.2% and 87.1±5.8%, respectively. None of the risk factors were significantly associated with the early mortality. Univariate analysis of risk factors showed that emergency surgery and re-surgery were significantly associated with late mortality (p<0.05), whereas multivariate analysis demonstrated that only emergency surgery (p<0.05) was significantly associated with late mortality. At 15 years, new aneursym, dissection-free and mitral regurgitation-free survival rates were 73.5±9.7%, 93.2±4.9%, 87.3±8.7%, respectively.

Conclusion: Our study results suggested improved survival associated with surgical intervention, while showing reduced life expectancy in the patients with Marfan syndrome who underwent emergency surgery. Therefore, those patients should be monitored for cardiovascular involvement carefully.