AORTIC COMPLICATIONS IN MARFAN SYNDROME. SHOULD WE ANTICIPATE PREVENTIVE AORTIC ROOT SURGERY

Abstract Background 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 (CT)/ magnetic resonance imaging (MRI) of aorta and periodic annual monitoring. Mean follow-up was 5.6±2.7 years. The annual incidence of aortic events was assessed according aortic diameters for the optimal time for prophylactic surgery to be decided upon. 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 (0.2%/year (CI:0.03-0.5) with diameters Conclusions The incidence of aortic events in patients with Marfan syndrome followed at specific units is low, allthough the risk increases with aortic diameters ≥ 45 mm. These results support current indications, but we consider it reasonable, at experienced centers (elective root aortic surgery mortality