Characteristics And Long-Term Outcomes In Patients With Marfan Syndrome: A Tertiary Care Center Experience

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, pu003c0.001). Data on longer-term multivariable Cox survival analysis performed in operated patients (n=535) demonstrated that prophylactic ascending aortic surgery ...