Abstract 17467: Characteristics and Long-Term Outcomes in Patients With Marfan Syndrome

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Abstract

Background: We sought to assess characteristics and longer-term outcomes of patients with Marfan syndrome (MFS) evaluated at a high-volume tertiary care center.

Methods: We included 749 MFS patients (≥18 years, all meet 2010 revised Ghent criteria) who presented at our tertiary center between 1990-2016. Patients were divided in 3 groups: 214 (28%) conservatively managed without advanced aortopathy (CM), 343 (46%) with prophylactic ascending aortic surgery (PS) and 192 (26%) with emergency surgery (ES) following initial Type A dissection. Primary endpoint was death and/or aortic dissection.

Results: Clinical data are shown in Figure 1. At 8±5 years follow up, there were 138 primary events (18%, 97 [13%] deaths and 62 [8%] dissections). In operated patients, 30-day mortality occurred in 12 (2%) patients (0.3% in PS and 6% in ES groups respectively, p<0.001). Data on longer-term multivariable Cox survival analysis performed in operated patients (n=535) demonstrated that prophylactic ascending aortic surgery was associated with improved longer-term event-free survival (Figure 2a). 1, 5 and 10-year freedom from events in the CM (100%, 98% and 97%), PS (98%, 93% and 85%) and ES (89%, 74% and 61%), respectively. Kaplan-Meier curves (Figure 2b) demonstrate that longer-term freedom from primary endpoint was significantly better in the PS vs. ES group (also, the CM group without advanced aortopathy had excellent longer-term outcomes).

Conclusion: In MFS with significant ascending aortopathy, prophylactic aortic surgery was associated with significantly improved survival and lesser rate of dissection when compared to emergency surgery following initial Type A aortic dissection.

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