40. Mapping of Ventricular Tachycardias in Rare Cardiomyopathies

  1. Mohammad Shenasa MD2,
  2. Gerhard Hindricks MD3,
  3. Martin Borggrefe MD4,
  4. Günter Breithardt MD5 and
  5. Mark E. Josephson MD6
  1. Lars Eckardt,
  2. Dirk Dechering,
  3. Stephan Zellerhoff,
  4. Günter Breithardt and
  5. Kristina Wasmer

Published Online: 18 DEC 2012

DOI: 10.1002/9781118481585.ch40

Cardiac Mapping, Fourth Edition

Cardiac Mapping, Fourth Edition

How to Cite

Eckardt, L., Dechering, D., Zellerhoff, S., Breithardt, G. and Wasmer, K. (2013) Mapping of Ventricular Tachycardias in Rare Cardiomyopathies, 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.ch40

Editor Information

  1. 2

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

  2. 3

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

  3. 4

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

  4. 5

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

  5. 6

    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. Hospital of the Westfälische Wilhelms – University of Münster, Münster, Germany

Publication History

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

ISBN Information

Print ISBN: 9780470670460

Online ISBN: 9781118481585



  • rare cardiomyopathies;
  • cardiac magnetic resonance imaging;
  • positron emission tomography;
  • ventricular tachyarrhythmias;
  • catheter ablation;
  • antiarrhythmic drugs;
  • cardiac sarcoidosis;
  • amyloidosis


Rare cardiomyopathies are a heterogeneous group of diseases that often present clinically with signs of heart failure and/or arrhythmias. Even in the era of new technology, such as positron emission tomography and cardiac magnetic resonance imaging (MRI), early diagnosis remains difficult. A feared complication, e.g., of sarcoidosis, is granulomatous myocardial infiltration forming a substrate for potentially life-threatening arrhythmias. Spontaneous ventricular tachyarrhythmias may be considered to be a manifestation of disease activity or inflammation. In many patients with rare cardiomyopathies, abnormal Holter findings may be the first sign of cardiac involvement. Others may be detected by a routine ECG or echocardiography. Importantly, sudden death may also be the initial manifestation. In contrast to post myocardial infarction ventricular tachycardias (VTs), arrhythmias in rare cardiomyopathies tend to be more difficult to control due to diffuse and heterogeneous ventricular involvement and potential deep intramural circuits. The diffuse myocardial involvement and the progressive nature of diseases may illustrate the challenge of mapping and ablation of ventricular tachyarrhythmias. The most common mechanism of these arrhythmias is re-entry due to slow conduction caused by increased myocardial fibrosis. Mapping and catheter ablation has been attempted in rare cardiomyopathy patients with frequent ventricular tachyarrhythmia episodes, but results have been disappointing. The success rate of VT ablation, e.g., in patients with cardiac sarcoidosis, was much lower than in patients with ischemic heart disease. In many patients with rare cardiomyopathies a combined approach to arrhythmia management using specific therapy such as immunosuppressive medications as in cardiac sarcoidosis, implantable devices and antiarrhythmic drugs and/or catheter ablation may be necessary. Mapping and ablation should be performed in patients with recurrent ventricular tachyarrhythmias, but in the vast majority of patients with rare cardiomyopathies catheter ablation remains an adjunct therapy to implantable cardioverter-defibrillator.