ABSTRACT
BACKGROUND: The purpose of this study was to analyze the risk of aortic events (death, dissection, or aortic rupture) associated with Marfan syndrome and decide on the optimal timing for preventive surgery on the aortic root. METHODS: From January 2004 to June 2015, 397 patients from Marfan Units were studied by echocardiographic and computed tomography and magnetic resonance imaging of aorta and periodic annual monitoring. Mean follow-up was 5.6 ± 2.7 years. The annual incidence of aortic events was assessed according to aortic diameters for the optimal time for prophylactic surgery to be decided on. RESULTS: Mean age at the first visit was 28.4 ± 14.5 years, with mean sinuses of Valsalva diameter of 37.1 ± 6.6 mm., Eleven aortic events occurred during follow-up: seven deaths and four acute aortic dissections. Mean annual risk of an aortic event was 0.5% (risk of death 0.32% and risk of aortic dissection 0.18%). The increase in aortic diameters was associated with increased risk of 0.2% per year (95% confidence interval [CI], 0.03 to 0.5) with diameters less than 40 mm, 0.3% per year (95% CI, 0.1 to 1.4) between 40 and 44 mm, 1.3% per year (95% CI, 0.3 to 4.6) between 45 and 49 mm, and 5.2% per year (95% CI, 0.4 to 12.4) with diameters 50 mm or greater. Fifty-six patients underwent elective surgery with no inhospital mortality. Overall survival at 3, 5, and 10 years was 99% ± 5%, 98.6% ± 6%, and 95.5% ± 2.5%, respectively. CONCLUSIONS: The incidence of aortic events in patients with Marfan syndrome followed at specific units is low, although the risk increases with aortic diameters of 45 mm or greater. These results support current indications, but we consider it reasonable at experienced centers (elective root aortic surgery mortality less than 1%) to indicate an early surgery when the aortic root exceeds 45 mm.
