Abstract
Patients with myotonic dystrophy, the most common neuromuscular dystrophy in adults, have a high prevalence of arrhythmic complications with increased cardiovascular mortality and high risk for sudden death. Sudden death prevention is central and relies on annual follow-up and prophylactic permanent pacing in patients with conduction defects on electrocardiogram and/or infrahisian blocks on electrophysiological study. Implantable cardiac defibrillator therapy may be indicated in patients with ventricular tachyarrhythmia.
Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.
MeSH terms
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Animals
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Arrhythmias, Cardiac / genetics
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Arrhythmias, Cardiac / mortality
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Arrhythmias, Cardiac / physiopathology
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Arrhythmias, Cardiac / therapy*
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Cardiac Pacing, Artificial* / adverse effects
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Cardiac Pacing, Artificial* / mortality
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Death, Sudden, Cardiac / epidemiology
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Death, Sudden, Cardiac / prevention & control*
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Defibrillators, Implantable
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Electric Countershock* / adverse effects
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Electric Countershock* / instrumentation
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Electric Countershock* / mortality
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Genetic Predisposition to Disease
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Humans
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Myotonic Dystrophy / epidemiology*
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Myotonic Dystrophy / genetics
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Myotonic Dystrophy / mortality
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Myotonic Dystrophy / physiopathology
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Pacemaker, Artificial
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Prevalence
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Risk Factors
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Treatment Outcome