Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study

Abstract Aim Muscular dystrophy (MD) is a progressive disease with predominantly muscular symptoms. Myotonic dystrophy type II (MD2) and facioscapulohumeral muscular dystrophy type 1 (FSHD1) are gaining an increasing awareness, but data on cardiac involvement are conflicting. The aim of this study w...

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Autores principales:	Edyta Blaszczyk, Carolin Lim, Peter Kellman, Luisa Schmacht, Jan Gröschel, Simone Spuler, Jeanette Schulz-Menger
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Magnetic Resonance Imaging Facioscapulohumeral muscular dystrophy type 1 Myotonic dystrophy type 2 Fat Fibrosis Remodeling Diseases of the circulatory (Cardiovascular) system RC666-701
Acceso en línea:	https://doaj.org/article/02def748032d443ba1a9fcc0f36017b5
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spelling	oai:doaj.org-article:02def748032d443ba1a9fcc0f36017b52021-11-14T12:12:47ZProgressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study10.1186/s12968-021-00812-61532-429Xhttps://doaj.org/article/02def748032d443ba1a9fcc0f36017b52021-11-01T00:00:00Zhttps://doi.org/10.1186/s12968-021-00812-6https://doaj.org/toc/1532-429XAbstract Aim Muscular dystrophy (MD) is a progressive disease with predominantly muscular symptoms. Myotonic dystrophy type II (MD2) and facioscapulohumeral muscular dystrophy type 1 (FSHD1) are gaining an increasing awareness, but data on cardiac involvement are conflicting. The aim of this study was to determine a progression of cardiac remodeling in both entities by applying cardiovascular magnetic resonance (CMR) and evaluate its potential relation to arrhythmias as well as to conduction abnormalities. Methods and results 83 MD2 and FSHD1 patients were followed. The participation was 87% in MD2 and 80% in FSHD1. 1.5 T CMR was performed to assess functional parameters as well as myocardial tissue characterization applying T1 and T2 mapping, fat/water-separated imaging and late gadolinium enhancement. Focal fibrosis was detected in 23% of MD2) and 33% of FSHD1 subjects and fat infiltration in 32% of MD2 and 28% of FSHD1 subjects, respectively. The incidence of all focal findings was higher at follow-up. T2 decreased, whereas native T1 remained stable. Global extracellular volume fraction (ECV) decreased similarly to the fibrosis volume while the total cell volume remained unchanged. All patients with focal fibrosis showed a significant increase in left ventricular (LV) and right ventricular (RV) volumes. An increase of arrhythmic events was observed. All patients with ventricular arrhythmias had focal myocardial changes and an increased volume of both ventricles (LV end-diastolic volume (EDV) p = 0.003, RVEDV p = 0.031). Patients with supraventricular tachycardias had a significantly higher left atrial volume (p = 0.047). Conclusion We observed a remarkably fast and progressive decline of cardiac morphology and function as well as a progression of rhythm disturbances, even in asymptomatic patients with a potential association between an increase in arrhythmias and progression of myocardial tissue damage, such as focal fibrosis and fat infiltration, exists. These results suggest that MD2 and FSHD1 patients should be carefully followed-up to identify early development of remodeling and potential risks for the development of further cardiac events even in the absence of symptoms. Trial registration ISRCTN, ID ISRCTN16491505. Registered 29 November 2017 – Retrospectively registered, http://www.isrctn.com/ISRCTN16491505Edyta BlaszczykCarolin LimPeter KellmanLuisa SchmachtJan GröschelSimone SpulerJeanette Schulz-MengerBMCarticleMagnetic Resonance ImagingFacioscapulohumeral muscular dystrophy type 1Myotonic dystrophy type 2FatFibrosisRemodelingDiseases of the circulatory (Cardiovascular) systemRC666-701ENJournal of Cardiovascular Magnetic Resonance, Vol 23, Iss 1, Pp 1-16 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Magnetic Resonance Imaging Facioscapulohumeral muscular dystrophy type 1 Myotonic dystrophy type 2 Fat Fibrosis Remodeling Diseases of the circulatory (Cardiovascular) system RC666-701
spellingShingle	Magnetic Resonance Imaging Facioscapulohumeral muscular dystrophy type 1 Myotonic dystrophy type 2 Fat Fibrosis Remodeling Diseases of the circulatory (Cardiovascular) system RC666-701 Edyta Blaszczyk Carolin Lim Peter Kellman Luisa Schmacht Jan Gröschel Simone Spuler Jeanette Schulz-Menger Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
description	Abstract Aim Muscular dystrophy (MD) is a progressive disease with predominantly muscular symptoms. Myotonic dystrophy type II (MD2) and facioscapulohumeral muscular dystrophy type 1 (FSHD1) are gaining an increasing awareness, but data on cardiac involvement are conflicting. The aim of this study was to determine a progression of cardiac remodeling in both entities by applying cardiovascular magnetic resonance (CMR) and evaluate its potential relation to arrhythmias as well as to conduction abnormalities. Methods and results 83 MD2 and FSHD1 patients were followed. The participation was 87% in MD2 and 80% in FSHD1. 1.5 T CMR was performed to assess functional parameters as well as myocardial tissue characterization applying T1 and T2 mapping, fat/water-separated imaging and late gadolinium enhancement. Focal fibrosis was detected in 23% of MD2) and 33% of FSHD1 subjects and fat infiltration in 32% of MD2 and 28% of FSHD1 subjects, respectively. The incidence of all focal findings was higher at follow-up. T2 decreased, whereas native T1 remained stable. Global extracellular volume fraction (ECV) decreased similarly to the fibrosis volume while the total cell volume remained unchanged. All patients with focal fibrosis showed a significant increase in left ventricular (LV) and right ventricular (RV) volumes. An increase of arrhythmic events was observed. All patients with ventricular arrhythmias had focal myocardial changes and an increased volume of both ventricles (LV end-diastolic volume (EDV) p = 0.003, RVEDV p = 0.031). Patients with supraventricular tachycardias had a significantly higher left atrial volume (p = 0.047). Conclusion We observed a remarkably fast and progressive decline of cardiac morphology and function as well as a progression of rhythm disturbances, even in asymptomatic patients with a potential association between an increase in arrhythmias and progression of myocardial tissue damage, such as focal fibrosis and fat infiltration, exists. These results suggest that MD2 and FSHD1 patients should be carefully followed-up to identify early development of remodeling and potential risks for the development of further cardiac events even in the absence of symptoms. Trial registration ISRCTN, ID ISRCTN16491505. Registered 29 November 2017 – Retrospectively registered, http://www.isrctn.com/ISRCTN16491505
format	article
author	Edyta Blaszczyk Carolin Lim Peter Kellman Luisa Schmacht Jan Gröschel Simone Spuler Jeanette Schulz-Menger
author_facet	Edyta Blaszczyk Carolin Lim Peter Kellman Luisa Schmacht Jan Gröschel Simone Spuler Jeanette Schulz-Menger
author_sort	Edyta Blaszczyk
title	Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
title_short	Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
title_full	Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
title_fullStr	Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
title_full_unstemmed	Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
title_sort	progressive myocardial injury in myotonic dystrophy type ii and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study
publisher	BMC
publishDate	2021
url	https://doaj.org/article/02def748032d443ba1a9fcc0f36017b5
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Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study

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