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JOURNAL ARTICLE

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

Carlos Martín, Arturo Evangelista, Santiago Serrano-Fiz, Susana Villar, Victor Ospina, Daniel Martínez, Juan De Villarreal, Violeta Sanchez, Vanessa Moñivas, Susana Mingo, Alberto Forteza
Annals of Thoracic Surgery 2019 October 4
31589859

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 <40 mm, 0.3%/year (CI 0.1-1, 4) between 40-44 mm, 1.3%/year (CI:0.3-4.6) between 45-49 mm and 5.2%/year (CI:0.4-12.4) with diameters ≥50 mm). Fifty-six patients underwent elective surgery with no in-hospital 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, 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 <1%), to indicate an early surgery when the aortic root exceeds 45 mm.

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