Cardiovascular Reoperations in Marfan Syndrome

Background And Aim Of The Study: The purpose of this study is to analyze the outcome results of reoperations in Marfan syndrome patients.

Methods: Between 1985 and December 2004, 49 patients with Marfan syndrome were operated for aortic aneurysms. Of these 49 patients, 9 (18,4%) required > or =1 reoperations after a mean duration of 32.2 +/- 26.6 months. The mean duration of follow-up was 52.0 +/- 46.8 months, a total of 39 patient/years. Survival free of reoperation was calculated by Cox regression analysis.

Results: Surgical indication for operation was a chronic aneurysmal dilatation of the ascending or abdominal aorta in seven patients (77.8%) and aortic dissection in two (22.2%) at the initial operation. In the reoperations, repair of thoracoabdominal aortic aneurysm with separated graft interposition in six patients (66.7%), replacement of ascending aorta in one (11.1%), replacement of ascending and hemiarchus aorta in one (11.1%), and mitral valve replacement in three patients (33.3%) were performed. The hospital mortality was 11.1% with one patient. Among the survivors, one expired in the follow-up five months after the second operation (12.5%). With the Cox regression analysis, survival without reoperation for 13, 24, and 123 months are 95.56 +/- 3.04%, 90.66 +/- 4.40%, and 60.32 +/- 12.63%, respectively. Mean survival for reoperated patients is 99 +/- 14 months (95% confidence interval 72-127 months).

Conclusions: Reoperations can be done with low morbidity and mortality. Patients should be kept under close follow-up using imaging techniques infinitely.