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Electrical Diseases of the Heart pp 170–186Cite as

Sodium Ion Channelopathies

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Abstract

Voltage-gated Sodium (Na+) channels are sarcolemmal proteins that are responsible for the rapid upstroke of the cardiac action potential (AP), and for rapid impulse conduction through cardiac tissue. Therefore, Na+ channel function plays a major role in initiation, propagation, and maintenance of the normal cardiac rhythm. Mutations in SCN5A, the gene encoding for the α-subunit of the cardiac Na+ channel, the so-called “inherited sodium channelopathies,” are known to evoke multiple life-threatening disorders of cardiac rhythm that can vary from tachyarrhythmias to bradyarrhythmias and may require implantation of pacemakers or implantable cardioverter/defibrillators (ICDs). However, recent studies have also linked Na+ current (INa) dysfunction to structural cardiac defects, notably cardiac fibrosis, dilated cardiomyopathy, and, possibly, arrhythmogenic right ventricular cardiomyopathy. These structural changes may also be conducive to (reentrant) arrhythmias.

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Authors and Affiliations

  1. Heart Failure Research Center Department of Clinical and Experimental Cardiology, Academic Medical Center, Amsterdam, The Netherlands

    Simona Casini MSc (PhD Student), Arthur A. M. Wilde MD, PhD, FESC, FAHA (Professor) & Hanno L. Tan MD, PhD (Cardiologist)

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  1. Simona Casini MSc
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  2. Arthur A. M. Wilde MD, PhD, FESC, FAHA
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  3. Hanno L. Tan MD, PhD
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Editor information

Editors and Affiliations

  1. Cardiovascular Boehringer Ingelheim Pharmaceuticals Ridgefield, CT, USA

    Ihor Gussak MD, PhD, FACC (Deputy Therapeutic Area Head, Professor of Pharmacology) (Deputy Therapeutic Area Head, Professor of Pharmacology)

  2. Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, Bridgewater, NJ, USA

    Ihor Gussak MD, PhD, FACC (Deputy Therapeutic Area Head, Professor of Pharmacology) (Deputy Therapeutic Area Head, Professor of Pharmacology)

  3. Gordon K. Moe Scholar Masonic Medical Research Laboratory, Utica, NY, USA

    Charles Antzelevitch PhD, FACC, FAHA, FHRS (Executive Director and Director of Research) (Executive Director and Director of Research)

  4. Upstate Medical University, Syracuse, NY, USA

    Charles Antzelevitch PhD, FACC, FAHA, FHRS (Executive Director and Director of Research) (Executive Director and Director of Research)

  5. Heart Failure Research Center Department of Clinical and Experimental Cardiology, Academic Medical Center, Amsterdam, The Netherlands

    Arthur A. M. Wilde MD, PhD, FESC, FAHA (Professor) (Professor)

  6. Mayo Clinic College of Medicine, Rochester, MN, USA

    Paul A. Friedman MD (Professor of Medicine) & Michael J. Ackerman MD, PhD, FACC (Professor of Medicine, Pediatrics and Pharmacology, Consultant Cardiovascular Disease and Pediatric Cardiology, Director Long QT Syndrome Clinic and the Mayo Clinic Windland Smith Rice Sudden Death Genomics) (Professor of Medicine) &  (Professor of Medicine, Pediatrics and Pharmacology, Consultant Cardiovascular Disease and Pediatric Cardiology, Director Long QT Syndrome Clinic and the Mayo Clinic Windland Smith Rice Sudden Death Genomics)

  7. Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, MN, USA

    Win-Kuang Shen MD (Consultant, Professor of Medicine) (Consultant, Professor of Medicine)

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© 2008 Springer-Verlag London Limited

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Casini, S., Wilde, A.A.M., Tan, H.L. (2008). Sodium Ion Channelopathies. In: Gussak, I., Antzelevitch, C., Wilde, A.A.M., Friedman, P.A., Ackerman, M.J., Shen, WK. (eds) Electrical Diseases of the Heart. Springer, London. https://doi.org/10.1007/978-1-84628-854-8_10

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  • DOI: https://doi.org/10.1007/978-1-84628-854-8_10

  • Publisher Name: Springer, London

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  • Online ISBN: 978-1-84628-854-8

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