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Cardiac involvement in the muscular dystrophies

Nicholas J Silvestri1, Haisam Ismail2, Peter Zimetbaum2

  • 1Department of Neurology, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, 1010 Main St Buffalo, New York, 14202, USA.

Muscle & Nerve
|November 14, 2017
PubMed

Insights

Cardiac disease is a frequent complication of muscular dystrophies, often presenting as cardiomyopathy or arrhythmias. Early monitoring and management are crucial for patients with these inherited muscle disorders to prevent sudden cardiac death.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Cardiac disease is a common manifestation in neuromuscular disorders, particularly muscular dystrophies.
  • Heart involvement may not be clinically apparent at initial presentation but can lead to severe complications.
  • Pathologic changes in the myocardium and conduction system cause cardiomyopathy and arrhythmias.

Purpose of the Study:

  • To review the spectrum of cardiac dysfunction in inherited muscle disorders.
  • To provide practical recommendations for monitoring and management of cardiac involvement.

Main Methods:

  • Literature review of cardiac involvement in muscular dystrophies.
  • Analysis of pathological changes in the myocardium and cardiac conduction system.
  • Synthesis of current monitoring and management strategies.

Main Results:

  • Cardiac dysfunction includes cardiomyopathy and rhythm disturbances (supraventricular and ventricular arrhythmias).
  • These can lead to life-threatening events such as sudden cardiac death.
  • Early identification and management are essential.

Conclusions:

  • Cardiac involvement is a significant concern in muscular dystrophies.
  • Regular cardiac monitoring and timely intervention are vital for improving patient outcomes.
  • This review offers guidance for clinicians managing these patients.

Keywords:
bundle branch blockcardiomyopathyimplantable cardioverter defibrillatormuscular dystrophysudden cardiac death

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