Definition and treatment of arrhythmogenic cardiomyopathy: an updated expert panel report

Eur J Heart Fail. 2019 Aug;21(8):955-964. doi: 10.1002/ejhf.1534. Epub 2019 Jun 18.


It is 35 years since the first description of arrhythmogenic right ventricular cardiomyopathy (ARVC) and more than 20 years since the first reports establishing desmosomal gene mutations as a major cause of the disease. Early advances in the understanding of the clinical, pathological and genetic architecture of ARVC resulted in consensus diagnostic criteria, which proved to be sensitive but not entirely specific for the disease. In more recent years, clinical and genetic data from families and the recognition of a much broader spectrum of structural disorders affecting both ventricles and associated with a propensity to ventricular arrhythmia have raised many questions about pathogenesis, disease terminology and clinical management. In this paper, we present the conclusions of an expert round table that aimed to summarise the current state of the art in arrhythmogenic cardiomyopathies and to define future research priorities.

Keywords: Arrhythmogenic cardiomyopathy; Arrhythmogenic right ventricular cardiomyopathy; Ventricular arrhythmias.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Anti-Arrhythmia Agents / therapeutic use*
  • Arrhythmogenic Right Ventricular Dysplasia / drug therapy*
  • Arrhythmogenic Right Ventricular Dysplasia / physiopathology
  • Consensus*
  • Electrocardiography
  • Humans


  • Anti-Arrhythmia Agents