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Cardiac Disease in Duchenne and Becker Muscular Dystrophies: The Dystrophinopathies

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Molecular Genetics of Cardiac Electrophysiology

Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 231))

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Abstract

The muscular dystrophies are a clinically and genetically heterogeneous group of skeletal muscle wasting diseases that differ widely in their frequency and pattern of cardiac involvement. Clinically, muscular dystrophies are characterized by progressive muscle weakness and atrophy. Pathologically, muscle histology reveals fiber size variability, central nuclei, as well as muscle necrosis with fatty replacement and fibrosis (1,2). Their pattern of muscle involvement, severity, mode of inheritance, unique clinical features, and primary gene defect distinguish individual types of muscular dystrophy (3).

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Author information

Authors and Affiliations

  1. Department of Cardiology, Children’s Hospital, Boston, USA

    Leslie B. Smoot M.D.

  2. Department of Pediatrics, Harvard Medical School, Boston, MA, 02115, USA

    Gerald Cox M.D.

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  1. Leslie B. Smoot M.D.
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  2. Gerald Cox M.D.
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Editors and Affiliations

  1. Department of Cardiology, Children’s Hospital, Boston, USA

    Charles I. Berul M.D.

  2. Department of Pediatrics, Harvard Medical School, Boston, 02115, MA, USA

    Charles I. Berul M.D.

  3. Departments of Pediatrics (Cardiology), Molecular and Human Genetics, Texas Children’s Hospital, Baylor College of Medicine, Houston, 77030, TX, USA

    Jeffrey A. Towbin M.D.

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Smoot, L.B., Cox, G. (2000). Cardiac Disease in Duchenne and Becker Muscular Dystrophies: The Dystrophinopathies. In: Berul, C.I., Towbin, J.A. (eds) Molecular Genetics of Cardiac Electrophysiology. Developments in Cardiovascular Medicine, vol 231. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-4517-0_16

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  • DOI: https://doi.org/10.1007/978-1-4615-4517-0_16

  • Publisher Name: Springer, Boston, MA

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