Cardiac assessment in duchenne and becker muscular dystrophies

Anitra Romfh, Elizabeth M. McNally

Research output: Contribution to journalReview articlepeer-review

73 Scopus citations

Abstract

Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophies. In addition to muscle disease, there nearly always is an associated cardiomyopathy in Duchenne or Becker muscular dystrophy. In these muscular dystrophies, the severity of cardiomyopathy and congestive heart failure may not parallel the severity of skeletal muscle disease. Loss of normal dystrophin function in the heart produces four-chamber dilation and reduction in left ventricular function that develop after the onset of muscle weakness. Arrhythmias affecting both atrial and ventricular rhythms occur and may be life threatening. The degree to which hypoventilation and pulmonary dysfunction are present also directly affect cardiac function in muscular dystrophy. Care guidelines recently were issued to outline surveillance and treatment strategies for the younger patient with Duchenne muscular dystrophy. Herein, we review those guidelines, and additionally, provide recommendations for monitoring and treating cardiac disease in the populations of advanced Duchenne and Becker muscular dystrophies.

Original languageEnglish (US)
Pages (from-to)212-218
Number of pages7
JournalCurrent Heart Failure Reports
Volume7
Issue number4
DOIs
StatePublished - Dec 1 2010

Keywords

  • Arrhythmia
  • Becker muscular dystrophy
  • Cardiac magnetic resonance
  • Cardiomyopathy
  • Congestive heart failure
  • Duchenne muscular dystrophy
  • Dystrophin
  • Late gadolinium enhancement
  • Muscular dystrophy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)
  • Emergency Medicine

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