59. Role of Mapping and Ablation in Genetic Diseases: Long QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia

  1. Mohammad Shenasa MD4,
  2. Gerhard Hindricks MD5,
  3. Martin Borggrefe MD6,
  4. Günter Breithardt MD7 and
  5. Mark E. Josephson MD8
  1. Steven J. Fowler1,
  2. Larry A. Chinitz1 and
  3. Silvia G. Priori1,2,3

Published Online: 18 DEC 2012

DOI: 10.1002/9781118481585.ch59

Cardiac Mapping, Fourth Edition

Cardiac Mapping, Fourth Edition

How to Cite

Fowler, S. J., Chinitz, L. A. and Priori, S. G. (2013) Role of Mapping and Ablation in Genetic Diseases: Long QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia, in Cardiac Mapping, Fourth Edition (eds M. Shenasa, G. Hindricks, M. Borggrefe, G. Breithardt and M. E. Josephson), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118481585.ch59

Editor Information

  1. 4

    Attending Physician, Department of Cardiovascular Services, O'Connor Hospital, Heart & Rhythm Medical Group, San Jose, California, USA

  2. 5

    Professor of Medicine (Cardiology), University Leipzig, Heart Center, Director, Department of Electrophysiology, Leipzig, Germany

  3. 6

    Professor of Medicine (Cardiology), Head, Department of Cardiology, Angiology and Pneumology, University Medical Center, Mannheim Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany

  4. 7

    Professor Emeritus of Medicine and Cardiology, Department of Cardiology and Angiology, Hospital of the University of Münster, Münster, Germany

  5. 8

    Chief, Cardiovascular Medicine Division, Director, Harvard-Thorndike Electrophysiology Institute and Arrhythmia Service, Beth Israel Deaconess Medical Center, Herman C. Dana Professor of Medicine, Harvard Medical School, Boston, Massachusetts, USA

Author Information

  1. 1

    NYU Langone Medical Center, New York, NY, USA

  2. 2

    IRCCS Fondazione Salvatore Maugeri, Pavia, Italy

  3. 3

    Università di Pavia, Pavia, Italy

Publication History

  1. Published Online: 18 DEC 2012
  2. Published Print: 10 JAN 2013

ISBN Information

Print ISBN: 9780470670460

Online ISBN: 9781118481585



  • genetic;
  • inherited;
  • disease;
  • long QT;
  • catecholaminergic;
  • ventricular arrhythmias;
  • mapping;
  • ablation


Management of patients with channelopathies represents a challenging field for the electrophysiologist because, given the absence of obvious structural abnormalities of the cardiac tissue, it is difficult to define paradigms for the use of mapping and ablation. Anecdotal reports and studies conducted in limited numbers of patients support the view that ablative procedures should be part of the clinical armamentarium used to treat ion channel disorders, yet conclusive data are far from being available. We believe that the nuanced nature of heritable arrhythmogenic disease presents distinct challenges to mapping and radiofrequency ablation; therefore, in the present chapter we review currently available studies as well as selected cases from our experience focusing on patients with long QT syndrome and catecholaminergic polymorphic ventricular tachycardia.