Survival and complication free survival in Marfan's syndrome: implicationsof current guidelines

Objective-To evaluate survival and complication free survival in patients w ith Marfan's syndrome and to assess the possible influence of recently revi sed guidelines for prophylactic aortic root replacement in these patients. Methods-130 patients who had been attending one institution over 14 years w ere evaluated. Kaplan-Meier analysis was performed in 125 patients who did not present with aortic root dissection as the first sign of Marfan's syndr ome, with the end points: death, aortic root dissection, and prophylactic a ortic root replacement after diagnosis. In the patients developing aortic r oot dissection, current guidelines for prophylactic aortic root replacement were retrospectively applied to investigate the number of dissections that could theoretically have been prevented. The guidelines were: (1) aortic r oot diameter greater than or equal to 55 mm, (2) positive family history of aortic disisections and aortic root diameter greater than or equal to 50 m m, and (3) aortic root growth 62 mm/year. Outcomes following emergency surg ery (15 patients) and prophylactic surgery of the aortic root (30 patients) were compared. Results-Five and 10 year survival after diagnosis was 95% and 88%, and the five and 10 year complication free survival was 78% and 66%, respectively. Thirteen patients developed dissection, 30 underwent prophylactic repair, a nd 82 had an uncomplicated course. Eleven dissections could theoretically h ave been prevented by application of the current guidelines. Five year surv ival following emergency and prophylactic repair of the aortic root was 51% , and 97%, respectively. Conclusions-Survival in the Marfan's syndrome in the past 14 years seems sa tisfactory; with application of current guidelines, it has probably even im proved. However, because of the high fatality rate in Marfan patients devel oping aortic root dissection, more extensive screening for Marfan's syndrom e and a search for additional risk factors are desirable.