Cardiac Pathology in Myotonic Dystrophy Type 1

Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children, is a multi-systemic disorder affecting skeletal, cardiac, and smooth muscles as well as neurologic, endocrine and other systems. This review is on the cardiac pathology associated with DM1. The heart i...

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Autores principales:	Mani S. Mahadevan, Ramesh S. Yadava, Mahua Mandal
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	myotonic dystrophy RNA toxicity cardiac pathology cardiac conduction triplet repeat mutation antisense oligonucleotides Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/c8e2ed8baa994aefb28e1cc42472abb0
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spelling	oai:doaj.org-article:c8e2ed8baa994aefb28e1cc42472abb02021-11-11T17:17:47ZCardiac Pathology in Myotonic Dystrophy Type 110.3390/ijms2221118741422-00671661-6596https://doaj.org/article/c8e2ed8baa994aefb28e1cc42472abb02021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/21/11874https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children, is a multi-systemic disorder affecting skeletal, cardiac, and smooth muscles as well as neurologic, endocrine and other systems. This review is on the cardiac pathology associated with DM1. The heart is one of the primary organs affected in DM1. Cardiac conduction defects are seen in up to 75% of adult DM1 cases and sudden death due to cardiac arrhythmias is one of the most common causes of death in DM1. Unfortunately, the pathogenesis of cardiac manifestations in DM1 is ill defined. In this review, we provide an overview of the history of cardiac studies in DM1, clinical manifestations, and pathology of the heart in DM1. This is followed by a discussion of emerging data about the utility of cardiac magnetic resonance imaging (CMR) as a biomarker for cardiac disease in DM1, and ends with a discussion on models of cardiac RNA toxicity in DM1 and recent clinical guidelines for cardiologic management of individuals with DM1.Mani S. MahadevanRamesh S. YadavaMahua MandalMDPI AGarticlemyotonic dystrophyRNA toxicitycardiac pathologycardiac conductiontriplet repeat mutationantisense oligonucleotidesBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 11874, p 11874 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	myotonic dystrophy RNA toxicity cardiac pathology cardiac conduction triplet repeat mutation antisense oligonucleotides Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	myotonic dystrophy RNA toxicity cardiac pathology cardiac conduction triplet repeat mutation antisense oligonucleotides Biology (General) QH301-705.5 Chemistry QD1-999 Mani S. Mahadevan Ramesh S. Yadava Mahua Mandal Cardiac Pathology in Myotonic Dystrophy Type 1
description	Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children, is a multi-systemic disorder affecting skeletal, cardiac, and smooth muscles as well as neurologic, endocrine and other systems. This review is on the cardiac pathology associated with DM1. The heart is one of the primary organs affected in DM1. Cardiac conduction defects are seen in up to 75% of adult DM1 cases and sudden death due to cardiac arrhythmias is one of the most common causes of death in DM1. Unfortunately, the pathogenesis of cardiac manifestations in DM1 is ill defined. In this review, we provide an overview of the history of cardiac studies in DM1, clinical manifestations, and pathology of the heart in DM1. This is followed by a discussion of emerging data about the utility of cardiac magnetic resonance imaging (CMR) as a biomarker for cardiac disease in DM1, and ends with a discussion on models of cardiac RNA toxicity in DM1 and recent clinical guidelines for cardiologic management of individuals with DM1.
format	article
author	Mani S. Mahadevan Ramesh S. Yadava Mahua Mandal
author_facet	Mani S. Mahadevan Ramesh S. Yadava Mahua Mandal
author_sort	Mani S. Mahadevan
title	Cardiac Pathology in Myotonic Dystrophy Type 1
title_short	Cardiac Pathology in Myotonic Dystrophy Type 1
title_full	Cardiac Pathology in Myotonic Dystrophy Type 1
title_fullStr	Cardiac Pathology in Myotonic Dystrophy Type 1
title_full_unstemmed	Cardiac Pathology in Myotonic Dystrophy Type 1
title_sort	cardiac pathology in myotonic dystrophy type 1
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/c8e2ed8baa994aefb28e1cc42472abb0
work_keys_str_mv	AT manismahadevan cardiacpathologyinmyotonicdystrophytype1 AT rameshsyadava cardiacpathologyinmyotonicdystrophytype1 AT mahuamandal cardiacpathologyinmyotonicdystrophytype1
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Cardiac Pathology in Myotonic Dystrophy Type 1

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