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

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...

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Autores principales: Mas-Stachurska, Aleksandra, Egea, Gustavo, de Bruin-Bon, Rianne, Rudenick, Paula, Sanchis, Laura, Bouma, Berto J., Mulder, Barbara J., Bijnens, Bart, Sitges, Marta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8338999/
https://www.ncbi.nlm.nih.gov/pubmed/34349174
http://dx.doi.org/10.1038/s41598-021-95263-5
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author Mas-Stachurska, Aleksandra
Egea, Gustavo
de Bruin-Bon, Rianne
Rudenick, Paula
Sanchis, Laura
Bouma, Berto J.
Mulder, Barbara J.
Bijnens, Bart
Sitges, Marta
author_facet Mas-Stachurska, Aleksandra
Egea, Gustavo
de Bruin-Bon, Rianne
Rudenick, Paula
Sanchis, Laura
Bouma, Berto J.
Mulder, Barbara J.
Bijnens, Bart
Sitges, Marta
author_sort Mas-Stachurska, Aleksandra
collection PubMed
description 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 Fbn1(C1039G/+) 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.
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spelling pubmed-83389992021-08-05 Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome Mas-Stachurska, Aleksandra Egea, Gustavo de Bruin-Bon, Rianne Rudenick, Paula Sanchis, Laura Bouma, Berto J. Mulder, Barbara J. Bijnens, Bart Sitges, Marta Sci Rep Article 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 Fbn1(C1039G/+) 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. Nature Publishing Group UK 2021-08-04 /pmc/articles/PMC8338999/ /pubmed/34349174 http://dx.doi.org/10.1038/s41598-021-95263-5 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/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/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Article
Mas-Stachurska, Aleksandra
Egea, Gustavo
de Bruin-Bon, Rianne
Rudenick, Paula
Sanchis, Laura
Bouma, Berto J.
Mulder, Barbara J.
Bijnens, Bart
Sitges, Marta
Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
title Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
title_full Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
title_fullStr Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
title_full_unstemmed Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
title_short Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
title_sort postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8338999/
https://www.ncbi.nlm.nih.gov/pubmed/34349174
http://dx.doi.org/10.1038/s41598-021-95263-5
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