Mas-Stachurska, AleksandraSiegert, Anna-Maria ElisaBatlle, MontserratGorbenko del Blanco, DaryaMeirelles, ThaynaRubies, CiraBonorino, FabioSerra-Peinado, CarlaBijnens, BartBaudin, JulioSitges Carreño, MartaMont Girbau, LluísGuasch i Casany, EduardEgea Guri, Gustavo2017-10-062017-10-062017-09-252047-9980https://hdl.handle.net/2445/116287BACKGROUND: Marfan syndrome (MF) leads to aortic root dilatation and a predisposition to aortic dissection, mitral valve prolapse, and primary and secondary cardiomyopathy. Overall, regular physical exercise is recommended for a healthy lifestyle, but dynamic sports are strongly discouraged in MF patients. Nonetheless, evidence supporting this recommendation is lacking. Therefore, we studied the role of long-term dynamic exercise of moderate intensity on the MF cardiovascular phenotype. METHODS AND RESULTS: In a transgenic mouse model of MF (Fbn1C1039G/+), 4-month-old wild-type and MF mice were subjected to training on a treadmill for 5 months; sedentary littermates served as controls for each group. Aortic and cardiac remodeling was assessed by echocardiography and histology. The 4-month-old MF mice showed aortic root dilatation, elastic lamina rupture, and tunica media fibrosis, as well as cardiac hypertrophy, left ventricular fibrosis, and intramyocardial vessel remodeling. Over the 5-month experimental period, aortic root dilation rate was significantly greater in the sedentary MF group, compared with the wild-type group (∆mm, 0.27±0.07 versus 0.13±0.02, respectively). Exercise significantly blunted the aortic root dilation rate in MF mice compared with sedentary MF littermates (∆mm, 0.10±0.04 versus 0.27±0.07, respectively). However, these 2 groups were indistinguishable by aortic root stiffness, tunica media fibrosis, and elastic lamina ruptures. In MF mice, exercise also produced cardiac hypertrophy regression without changes in left ventricular fibrosis. CONCLUSIONS: Our results in a transgenic mouse model of MF indicate that moderate dynamic exercise mitigates the progression of the MF cardiovascular phenotype.26 p.application/pdfengcc-by-nc (c) Mas-Stachurska, Aleksandra et al., 2017http://creativecommons.org/licenses/by-nc/3.0/esMalalties hereditàriesExerciciAneurismes aòrticsModels animals en la investigacióGenetic diseasesExerciseAortic aneurysmsAnimal models in researchinfo:eu-repo/semantics/article6736232017-10-06info:eu-repo/semantics/openAccess28947563