Case Reports
doi: 10.1016/j.cjca.2012.01.012.
Epub 2012 Apr 4.
Emery-Dreifuss humeroperoneal muscular dystrophy: cardiac manifestations
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- PMID: 22480903
- DOI: 10.1016/j.cjca.2012.01.012
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Case Reports
Emery-Dreifuss humeroperoneal muscular dystrophy: cardiac manifestations
Can J Cardiol.
2012 Jul-Aug.
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.
Copyright © 2012 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
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