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Provocative Testing in Inherited Arrhythmias

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Electrical Diseases of the Heart

Abstract

Heritable channelopathies that include congenital long QT syndrome (LQTS), Brugada syndrome (BrS), and catecholaminergic polymorphic ven tricular tachycardia (CPVT) affect an estimated 1 in 2000 persons, may present with syncope or sudden cardiac death, and often elude detection by standard 12-lead electrocardiography (ECG). In LQTS, an estimated 40% of genetically affected subjects have “concealed” LQTS with a normal or borderline heart rate corrected QT interval (QTc) at rest. A significant proportion of patients with BrS has concealed BrS with no evidence of a type 1 Brugada electrocardiographic pattern at rest. Every patient with CPVT has a normal resting ECG. Provocative testing with catecholamines and pharmacological testing with sodium channel blockers are critical diagnostic tests in the evaluation of these channelopathies and can help unmask LQTS, BrS, and CPVT in their concealed state. The role of these provocative tests in the evaluation of inherited arrhythmia syndromes will be reviewed in this chapter.

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Author information

Authors and Affiliations

  1. Division of Cardiology Department of Internal Medicine, National Cardiovascular Center, Suita, Osaka, Japan

    Wataru Shimizu MD, PhD (Senior Staff Co-Director of Clinical Cardiac Electrophysiology Laboratory)

  2. Sudden Arrhythmia Death Syndromes (SADS) Foundation, 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)

Authors
  1. Wataru Shimizu MD, PhD
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  2. Michael J. Ackerman MD, PhD, FACC
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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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Shimizu, W., Ackerman, M.J. (2008). Provocative Testing in Inherited Arrhythmias. 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_29

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  • DOI: https://doi.org/10.1007/978-1-84628-854-8_29

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84628-853-1

  • Online ISBN: 978-1-84628-854-8

  • eBook Packages: MedicineMedicine (R0)

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