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Clinical Evaluation, Risk Stratification, and Management of Congenital Long QT Syndrome

  • Chapter
Cardiac Repolarization

Part of the book series: Contemporary Cardiology ((CONCARD))

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Abstract

Once considered an extremely rare and lethal arrhythmogenic peculiarity, the congenital long QT syndrome (LQTS) is understood today as a primary cardiac channelopathy that is far more common but less commonly lethal than previously recognized. The molecular breakthroughs of the 1990s led by the research laboratories of Drs. Mark Keating, Jeff Towbin, Silvia Priori, and others revealed the fundamental underpinnings of LQTS. Now, hundreds of mutations scattered amongst five cardiac channel genes account for approx two—thirds of LQTS. Further, LQTS is a “Rosetta stone,” providing an important molecular model for ventricular arrhythmogenesis. Despite these tremendous advances, the bench—top discoveries have not yet translated to the patient’ s bedside in the form of a standard, routine molecular diagnostic test.

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Authors
  1. Anant Khositseth MD
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  2. Michael J. Ackerman MD, PhD
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Editor information

Editors and Affiliations

  1. eResearch Technology Inc., Bridgewater, NJ, USA

    Ihor Gussak MD, PhD

  2. Masonic Medical Research Laboratory, Utica, NY, USA

    Charles Antzelevitch PhD

  3. Mayo Clinic, Rochester, MN, USA

    Stephen C. Hammill MD  & Win-Kuang Shen MD  & 

  4. St. Louis University Health Sciences Center, St. Louis, MO, USA

    Preben Bjerregaard MD, DMSc

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Khositseth, A., Ackerman, M.J. (2003). Clinical Evaluation, Risk Stratification, and Management of Congenital Long QT Syndrome. In: Gussak, I., Antzelevitch, C., Hammill, S.C., Shen, WK., Bjerregaard, P. (eds) Cardiac Repolarization. Contemporary Cardiology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-362-0_20

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  • DOI: https://doi.org/10.1007/978-1-59259-362-0_20

  • Publisher Name: Humana Press, Totowa, NJ

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