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Congenital Long QT Syndrome

  • Chapter
Electrical Diseases of the Heart

Abstract

Once considered an extremely rare yet lethal arrhythmogenic peculiarity, congenital long QT syndrome (LQTS) is understood today as a primary cardiac arrhythmia syndrome (“cardiac channelopathy”) that is both far more common and, overall, much less lethal than previously recognized. Clinically, LQTS is often characterized by prolongation of the heart rate corrected QT interval (QTc) on a 12-lead surface electrocardiogram (ECG) and is associated with syncope, seizures, and sudden cardiac death due to ventricular arrhythmias (Torsade des pointes, TdP) usually following a precipitating event such as exertion, extreme emotion, or auditory stimulation. The molecular breakthroughs of the 1990s, led in large measure by the research laboratories of Drs. Mark Keating and Jeffrey Towbin in conjunction with LQTS registries containing meticulously phenotyped patients directed by Drs. Arthur Moss and Peter Schwartz, revealed the fundamental molecular underpinnings of LQTS— namely, defective cardiac channels.1

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Authors and Affiliations

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

    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 Laboratory, President Sudden Arrhythmia Death Syndromes (SADS) Foundation)

  2. Department of Pediatrics Division of Pediatric Cardiology Faculty of Medicine, Ramathibodi Hospital Mahidol University, Bangkok, Thailand

    Anant Khositseth MD

  3. Departments of Medicine, Pediatrics, and Molecular Pharmacology and Experimental Therapeutics Divisions of Cardiovascular Diseases and Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN, USA

    David J. Tester BS (Senior Research Technologist II)

  4. Department of Cardiology, University of Pavia and IRCCS Policlinico San Matteo, Pavia, Italy

    Peter J. Schwartz MD (Professor of Cardiology)

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  1. Michael J. Ackerman MD, PhD, FACC
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  2. Anant Khositseth MD
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  3. David J. Tester BS
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  4. Peter J. Schwartz MD
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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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Ackerman, M.J., Khositseth, A., Tester, D.J., Schwartz, P.J. (2008). Congenital Long QT Syndrome. 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_33

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  • DOI: https://doi.org/10.1007/978-1-84628-854-8_33

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84628-853-1

  • Online ISBN: 978-1-84628-854-8

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