Emery-Dreifuss Humeroperoneal Muscular Dystrophy: Cardiac Manifestations

descriptionPublicationkeyboard_double_arrow_right Article 01 Jul 2012 Australia English Publisher:Elsevier BVJournal:Canadian Journal of Cardiology, volume 28, pages 5,160-516,000 (issn: 0828-282X,

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Authors: Parmar, Malvinder S.; Parmar, Kamalpreet S.;

doi: 10.1016/j.cjca.2012.01.012

pmid: 22480903

Emery-Dreifuss Humeroperoneal Muscular Dystrophy: Cardiac Manifestations

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Abstract

Emery-Dreifuss muscular dystrophy (EDMD) is an inherited disorder affecting skeletal and cardiac muscles and characterized by muscular atrophy, contractures, and cardiomyopathy with conduction defects. It can be X-linked or autosomal. Not all patients with EDMD develop heart involvement, but heart disease associated with EDMD can be unpredictable and may be life threatening. In rare cases heart problems may be the first symptom of EDMD. Early recognition of heart involvement is of utmost importance as placement of a pacemaker and/or defibrillator may be lifesaving.

Country

Australia

Related Organizations

University of Queensland
Australia
University of Queensland
Australia
University of Queensland
Australia
Northern Ontario School of Medicine
Canada

Keywords

Sudden-death, Adult, Male, Electromyography, DNA Mutational Analysis, Signal Processing, Computer-Assisted, 2705 Cardiology and Cardiovascular Medicine, Muscular Dystrophy, Emery-Dreifuss, Electrocardiography, Echocardiography, Bradycardia, Humans, Atrioventricular Block, Cardiomyopathies

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