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

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 m...

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Autores principales:	Aleksandra Mas-Stachurska, Gustavo Egea, Rianne de Bruin-Bon, Paula Rudenick, Laura Sanchis, Berto J. Bouma, Barbara J. Mulder, Bart Bijnens, Marta Sitges
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/b0f692e82db944b9b8321f9cfaf42029
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spelling	oai:doaj.org-article:b0f692e82db944b9b8321f9cfaf420292021-12-02T17:06:10ZPostsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome10.1038/s41598-021-95263-52045-2322https://doaj.org/article/b0f692e82db944b9b8321f9cfaf420292021-08-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-95263-5https://doaj.org/toc/2045-2322Abstract 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.Aleksandra Mas-StachurskaGustavo EgeaRianne de Bruin-BonPaula RudenickLaura SanchisBerto J. BoumaBarbara J. MulderBart BijnensMarta SitgesNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-10 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Aleksandra Mas-Stachurska Gustavo Egea Rianne de Bruin-Bon Paula Rudenick Laura Sanchis Berto J. Bouma Barbara J. Mulder Bart Bijnens Marta Sitges Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
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 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.
format	article
author	Aleksandra Mas-Stachurska Gustavo Egea Rianne de Bruin-Bon Paula Rudenick Laura Sanchis Berto J. Bouma Barbara J. Mulder Bart Bijnens Marta Sitges
author_facet	Aleksandra Mas-Stachurska Gustavo Egea Rianne de Bruin-Bon Paula Rudenick Laura Sanchis Berto J. Bouma Barbara J. Mulder Bart Bijnens Marta Sitges
author_sort	Aleksandra Mas-Stachurska
title	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_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_sort	postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/b0f692e82db944b9b8321f9cfaf42029
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Postsystolic thickening is a potential new clinical sign of injured myocardium in marfan syndrome

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