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Cardiovascular Manifestations of Myotonic Dystrophy

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Cardiovascular Genetics and Genomics

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Abstract

The myotonic dystrophies type 1 and type 2 are the most frequently inherited neuromuscular diseases of adult life. They are progressive multisystem disorders and predominantly affect the conduction system of the heart. Tachy and bradyarrhythmias are the predominant cardiac manifestation and are associated with up to 30% of all cause mortality in DM. Routine cardiac screening is recommended in all patients with a minimum of a yearly ECG, ECHO and 24 h Holter monitoring. In our practice we recommend electrophysiological studies when there are any abnormalities on the ECG. Future work will aim to define criteria for implantation of pacemakers and implantable cardioverter defibrillators.

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Author information

Authors and Affiliations

  1. MRC Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, London, UK

    Umesh Vivekananda

  2. Department of Clinical Neurology, UCLH National Hospital for Neurology and Neurosurgery, London, UK

    Michael Hanna

  3. Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK

    Chris Turner

Authors
  1. Umesh Vivekananda
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  2. Michael Hanna
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  3. Chris Turner
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Corresponding author

Correspondence to Chris Turner .

Editor information

Editors and Affiliations

  1. Institute of Cancer & Genetics, University Hospital of Wales, Cardiff, United Kingdom

    Dhavendra Kumar

  2. Barts Heart Centre, St Bartholomew’s Hospital, London, United Kingdom

    Perry Elliott

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Vivekananda, U., Hanna, M., Turner, C. (2018). Cardiovascular Manifestations of Myotonic Dystrophy. In: Kumar, D., Elliott, P. (eds) Cardiovascular Genetics and Genomics. Springer, Cham. https://doi.org/10.1007/978-3-319-66114-8_18

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  • DOI: https://doi.org/10.1007/978-3-319-66114-8_18

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-66112-4

  • Online ISBN: 978-3-319-66114-8

  • eBook Packages: MedicineMedicine (R0)

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