Cardiovascular manifestations of myotonic dystrophy

Trends Cardiovasc Med. 2020 May;30(4):232-238. doi: 10.1016/j.tcm.2019.06.001. Epub 2019 Jun 13.

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.

Publication types

  • Review

MeSH terms

  • Animals
  • Arrhythmias, Cardiac / genetics
  • Arrhythmias, Cardiac / mortality
  • Arrhythmias, Cardiac / physiopathology
  • Arrhythmias, Cardiac / therapy*
  • Cardiac Pacing, Artificial* / adverse effects
  • Cardiac Pacing, Artificial* / mortality
  • Death, Sudden, Cardiac / epidemiology
  • Death, Sudden, Cardiac / prevention & control*
  • Defibrillators, Implantable
  • Electric Countershock* / adverse effects
  • Electric Countershock* / instrumentation
  • Electric Countershock* / mortality
  • Genetic Predisposition to Disease
  • Humans
  • Myotonic Dystrophy / epidemiology*
  • Myotonic Dystrophy / genetics
  • Myotonic Dystrophy / mortality
  • Myotonic Dystrophy / physiopathology
  • Pacemaker, Artificial
  • Prevalence
  • Risk Factors
  • Treatment Outcome