Aortic Complications in Marfan Syndrome: Should We Anticipate Preventive Aortic Root Surgery?

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 Material and MethodsThis multicenter, retrospective, observational study was conducted in MS patients, who fulfilled the Ghent international criteria (genetic study if appropriate). All patients were recruited at the

2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines

“…3,4,10 However, the risk of aortic dissection increases when the aortic diameter is >5.0 cm and is greater in patients with a family history of aortic dissection or rapid aortic growth. 3,4,10…”

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“…Reconstructive surgery options are valve-sparing aortic root replacement or valved conduit aortic root replacement (Pepper et al, 2020). Indications for surgery are aortic diameter of more than 50 mm or more than 45 mm with concurrent risks and a growth rate of more than 5 mm/year (Goyal et al, 2017;Martín et al, 2020). However, research shows that not only the diameter is a predictor for the dissection and it should not be the sole indicator for intervention (Wenzel et al, 2021).…”

Association between the sinus of Valsalva height and biometric parameters in patients with Marfan syndrome associated with aortic root aneurysm

“…Patients with Marfan syndrome suf-fer from cardiovascular pathologies (Goyal et al, 2017;Grewal and Gittenberger-de Groot, 2018;Xuan et al, 2021). Aortic pathologies are reported in 90% of cases (Martín et al, 2020;Vanem et al, 2018). One of the forms of pathologies is the dilatation of the sinuses of Valsalva, which results in an aortic root aneurysm.…”

“…Reconstructive surgery options are valve-sparing aortic root replacement or valved conduit aortic root replacement (Pepper et al, 2020). Indications for surgery are aortic diameter of more than 50 mm or more than 45 mm with concurrent risks and a growth rate of more than 5 mm/year (Goyal et al, 2017;Martín et al, 2020). However, research shows that not only the diameter is a predictor for the dissection and it should not be the sole indicator for intervention (Wenzel et al, 2021).…”