Thoracic aortopathy in Marfan syndrome overlaps with mechanisms seen in bicuspid aortic valve disease

Autor: Nimrat Grewal, Onur Dolmaci, Evert Jansen, Robert Klautz, Antoine Driessen, Robert E. Poelmann
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Frontiers in Cardiovascular Medicine, Vol 10 (2023)
Druh dokumentu: article
ISSN: 2297-055X
DOI: 10.3389/fcvm.2023.1018167
Popis: BackgroundThoracic aortopathy is a serious complication which is more often seen in patients with Marfan syndrome (MFS) and patients with a bicuspid aortic valve (BAV) than in individuals with a tricuspid aortic valve (TAV). The identification of common pathological mechanisms leading to aortic complications in non-syndromic and syndromic diseases would significantly improve the field of personalized medicine.ObjectiveThis study sought to compare thoracic aortopathy between MFS, BAV, and TAV individuals.Materials and methodsBicuspid aortic valve (BAV; n = 36), TAV (n = 23), and MFS (n = 8) patients were included. Ascending aortic wall specimen were studied for general histologic features, apoptosis, markers of cardiovascular ageing, expression of synthetic and contractile vascular smooth muscle cells (VSMC), and fibrillin-1 expression.ResultsThe MFS group showed many similarities with the dilated BAV. Both patient groups showed a thinner intima (p
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