Arrhythmias in the muscular dystrophies

Research output: Contribution to journalArticle

59 Citations (Scopus)

Abstract

The muscular dystrophies are a group of inherited diseases affecting skeletal muscle that also affect cardiac muscle. Cardiac involvement occurs as a degenerative process with fibrosis and fatty replacement of the myocardium. Electrophysiologists are asked to participate in the care of muscular dystrophy patients because of the risk of atrial arrhythmias, conduction disease, bradycardia, ventricular arrhythmias, and sudden death. Duchenne, Becker, and limb-girdle types 2C-2F and 2I are muscular dystrophies in which the development of a dilated cardiomyopathy is common. Arrhythmias and conduction disease occur after the development of the dilated cardiomyopathy. Patients are considered for pacemakers or implantable cardioverter-defibrillators on the basis of guidelines used for nonischemic cardiomyopathies. Myotonic types 1 and 2, Emery-Dreifuss, limb-girdle type 1B, and facioscapulohumeral muscular dystrophies present with conduction disease and associated arrhythmias and variably with a dilated cardiomyopathy. In myotonic type 1, Emery-Dreifuss, and limb-girdle type 1B muscular dystrophies, conduction abnormalities are frequent and often require pacing. Recent studies support the use of prophylactic implantable cardioverter-defibrillators rather than pacemakers. In all the muscular dystrophies, respiratory muscle involvement can impact quality and quantity of life and needs to be factored in when considering a prophylactic device.

Original languageEnglish
Pages (from-to)1890-1895
Number of pages6
JournalHeart Rhythm
Volume9
Issue number11
DOIs
StatePublished - Nov 2012

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Muscular Dystrophies
Cardiac Arrhythmias
Dilated Cardiomyopathy
Implantable Defibrillators
Myocardium
Respiratory Muscles
Bradycardia
Sudden Death
Cardiomyopathies
Skeletal Muscle
Fibrosis
Extremities
Quality of Life
Guidelines
Equipment and Supplies
Type 1B Limb-girdle muscular dystrophy

Keywords

  • Arrhythmia
  • Cardiomyopathy
  • Genetics
  • Implantable cardioverter-defibrillator
  • Muscular dystrophy
  • Pacemaker
  • Sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Arrhythmias in the muscular dystrophies. / Groh, William.

In: Heart Rhythm, Vol. 9, No. 11, 11.2012, p. 1890-1895.

Research output: Contribution to journalArticle

Groh, William. / Arrhythmias in the muscular dystrophies. In: Heart Rhythm. 2012 ; Vol. 9, No. 11. pp. 1890-1895.
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