Arrhythmogenic right ventricular dysplasia/cardiomyopathy: an electrocardiogram-based review

Europace. 2018 Jun 1;20(FI1):f3-f12. doi: 10.1093/europace/eux202.


Arrhythmogenic right ventricular dysplasia or cardiomyopathy (ARVD/C) is a pathologic condition where the right ventricle is partially or totally replaced by fatty and fibrous tissue. The electrocardiogram (ECG) has a central role for diagnosis since it comprises two major and two minor criteria in the diagnostic criteria published by the Task 1 Force, although it is not 100% necessary to make a final diagnosis, because around 10% of patients with ARVD/C present with a normal ECG. In this article, we review the 12-lead electrocardiographic findings of patients with ARVD/C. ECG criteria observed during depolarization [prolonged terminal activation duration, epsilon wave, partial right bundle branch block (RBBB) and advanced RBBB of peripheral origin with characteristic ECG patterns] and repolarization abnormalities, (negative T waves) are reviewed in detail. More common ventricular arrhythmias and risk of sudden death, and how to use the surface ECG to stratify the risk, are part of our final comments.

Publication types

  • Review

MeSH terms

  • Action Potentials*
  • Arrhythmogenic Right Ventricular Dysplasia / diagnosis*
  • Arrhythmogenic Right Ventricular Dysplasia / mortality
  • Arrhythmogenic Right Ventricular Dysplasia / physiopathology
  • Arrhythmogenic Right Ventricular Dysplasia / therapy
  • Death, Sudden, Cardiac / epidemiology
  • Diagnosis, Differential
  • Electrocardiography*
  • Heart Rate*
  • Heart Ventricles / pathology
  • Heart Ventricles / physiopathology*
  • Humans
  • Predictive Value of Tests
  • Prognosis
  • Risk Assessment
  • Risk Factors