Objective: Patients with Marfan syndrome (MFS) and patients with a bicuspid aortic valve (BAV) have a significantly increased risk to develop thoracic aortopathy. Both conditions share many... Show moreObjective: Patients with Marfan syndrome (MFS) and patients with a bicuspid aortic valve (BAV) have a significantly increased risk to develop thoracic aortopathy. Both conditions share many pathophysiological mechanisms leading to aortic complications. Bicuspidy is known to have a low risk for acquired coronary artery sclerosis. The aim of this study is to determine the risk of coronary sclerosis in MFS patients. Methods: Marfan syndrome patients with an aortic root dilatation, which were surgically treated between 1999 and 2017, were included and matched with BAV and tricuspid aortic valves (TAV) patients based on sex and age. Cardiovascular risk profiles were determined in all three groups. Coronary sclerosis was graded in all patients on coronary imaging (coronary angiography or computed tomography) using a coronary artery scoring method, which divides the coronaries in 28 segments and scores non-obstructive (20-49% sclerosis) and obstructive coronary sclerosis (>49% sclerosis) in each segment. Results: A total of 90 matched patients (30 within each group) were included. MFS patients showed less cardiovascular risk factors compared to BAV and TAV patients. TAV patients had higher amounts of obstructive coronary sclerosis as compared to BAV patients (p = 0.039) and MFS patients (p = 0.032). No difference in non- and obstructive coronary artery disease (CAD) was found between the MFS and BAV population. Conclusion: Marfan syndrome and bicuspid aortic valve patients have a significantly lower risk for, and prevalence of CAD as compared to TAV individuals. Show less
Heer, F. de; Kluin, J.; Elkhoury, G.; Jondeau, G.; Enriquez-Sarano, M.; Schafers, H.J.; ... ; Aortic Valve Repair Res Network In 2019
Objectives: Current national registries are lacking detailed pathology-driven analysis and long-term patients outcomes. The Heart Valve Society (HVS) aortic valve (AV) repair research network... Show moreObjectives: Current national registries are lacking detailed pathology-driven analysis and long-term patients outcomes. The Heart Valve Society (HVS) aortic valve (AV) repair research network started the Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry (AVIATOR) to evaluate long-term patient outcomes of AV repair and replacement. The purpose of the current report is to describe the AVIATOR initiative and report in a descriptive manner the patients included.Methods: The AV repair research network includes surgeons, cardiologists, and scientists and established an online database compliant with the guidelines for reporting valve-related events. Prospective inclusion started from January 2013. Adult patients (18 years or older) who were operated on between 1995 and 2017 with complete procedural specification of the type of repair/replacement were selected for descriptive analysis.Results: Currently 58 centers from 17 countries include 4896 patients with 89% AV repair (n = 4379) versus 11% AV replacement (n = 517). AV repair was either isolated (28%), or associated with tubular/partial root replacement (22%) or valve-sparing root replacement (49%) with an in-hospital mortality of 0.5%, 1.7%, and 1.2%, respectively. AV replacement was either isolated (24%), associated with tubular/partial root replacement (17%) or root replacement (59%) with an in-hospital mortality of 1%, 2.6%, and 2.0%, respectively.Conclusions: The multicenter surgical AVIATOR registry, by applying uniform definitions, should provide a solid evidence base to evaluate the place of repair versus replacement on the basis of long-term patient outcomes. Obtaining data completeness and adequate representation of all surgery types remain challenging. Toward the near future AVIATOR-medical will start to study natural history, as will AVIATOR-kids, with a focus on pediatric disease. Show less
