Hereditary muscular dystrophies and the heart

M. C. E. Hermans*, Y. M. Pinto, Ingemar S. J. Merkies, C. E. M. de Die-Smulders, H. J. G. M. Crijns, C. G. Faber

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

107 Citations (Web of Science)

Abstract

Cardiac disease is a common clinical manifestation of neuromuscular disorders, particularly of muscular dystrophies. Heart muscle cells as well as specialized conducting myocardial fibres may be affected by the dystrophic process. The incidence and nature of cardiac involvement vary with different types of muscular dystrophies. Some mainly lead to myocardial disease, resulting in cardiomyopathy and heart failure, while others particularly affect the conduction system, leading to arrhythmias and sudden death. As prognosis of muscular dystrophy patients may be directly related to cardiac status, surveillance and timely management of cardiac complications are important. However, recognition of cardiac involvement requires active investigation and remains challenging since typical signs and symptoms of cardiac dysfunction may not be present and progression is unpredictable. In this review, we present a comprehensive overview of hereditary muscular dystrophies associated with cardiac disease to provide an efficient strategy for the expertise and management of these diseases.
Original languageEnglish
Pages (from-to)479-492
JournalNeuromuscular Disorders
Volume20
Issue number8
DOIs
Publication statusPublished - Aug 2010

Keywords

  • Muscular dystrophy
  • Cardiomyopathy
  • Arrhythmia
  • Sudden death
  • Treatment

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