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Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome

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dc.contributor.author Mas Stachurska, Aleksandra
dc.contributor.author Egea, Gustavo
dc.contributor.author de Bruin-Bon, Rianne
dc.contributor.author Rudenick, Paula A.
dc.contributor.author Sanchis, Laura
dc.contributor.author Bouma, Berto J.
dc.contributor.author Mulder, Barbara J.
dc.contributor.author Bijnens, Bart
dc.contributor.author Sitges, Marta
dc.date.accessioned 2022-07-11T06:50:33Z
dc.date.available 2022-07-11T06:50:33Z
dc.date.issued 2021
dc.identifier.citation Mas-Stachurska A, Egea G, de Bruin-Bon R, Rudenick P, Sanchis L, Bouma BJ, et al. Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome. Sci Rep. 2021 Aug 4; 11(1): 15790. DOI: 10.1038/s41598-021-95263-5
dc.identifier.issn 2045-2322
dc.identifier.uri http://hdl.handle.net/10230/53705
dc.description.abstract The mechanisms leading to cardiac remodeling in Marfan syndrome (MFS) are a matter of debate since it could be either due to structural dysfunction of the myocardial extracellular matrix or to increased afterload caused by the dilated aorta. We aim to characterize the presence of abnormal myocardial function in MFS and to investigate its potential association with increased afterload. Aorta, left ventricle (LV) and the postsystolic thickening (PST) were analyzed in echocardiography in Fbn1C1039G/+ mice and in patients with MFS in comparison with wild type (WT) mice and healthy humans. PST was more frequent in MFS than in WT mice (p < 0.05). MFS mice with PST showed larger aorta than those without PST. Patients with MFS showed larger aorta, poorer LV function and a higher prevalence of PST (56%) than did the healthy controls (23%); p = 0.003. Blood pressure was similar. The higher prevalence of PST in an experimental murine model and in MFS patients, regardless of systemic arterial pressure, suggests an increased afterload on the LV myocardium. This finding supports the use of PST as an indicator of myocardial damage and encourage searching for novel early preventive therapy.
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Nature Research
dc.rights Copyright © The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.
dc.rights.uri http://creativecommons.org/licenses/by/4.0/
dc.subject.other Cardiologia
dc.subject.other Cor--Malalties
dc.subject.other Marfan, síndrome de
dc.title Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.1038/s41598-021-95263-5
dc.relation.projectID info:eu-repo/grantAgreement/ES/2PE/SAF2017-83039-R
dc.rights.accessRights info:eu-repo/semantics/openAccess
dc.type.version info:eu-repo/semantics/publishedVersion


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