Diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy

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For the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) to be made, patients must have either two major criteria, one major and two minor criteria, or four minor criteria.

Major criteria

  • global or regional dysfunction and structural alterations:
    • severe dilatation of the right ventricle (RV) and reduced RV ejection fraction
    • severe segmental dilatation of the RV
    • localized RV aneurysm
  • tissue characterization:
    • fibrofatty replacement of the RV myocardium (endocardial biopsy)
  • depolarization or conduction abnormalities:
    • epsilon waves
    • prolonged QRS complex (>110msec) in V1-V3
  • family history:
    • familial disease confirmed at autopsy or surgery

Minor criteria

  • global or regional dysfunction and structural alterations:
    • mild dilatation of the right ventricle (RV) and reduced RV ejection fraction
    • mild segmental dilatation of the RV
    • regional RV hypokinesia
  • repolarization abnormalities:
    • inverted T waves (V2-V3)
  • depolarization or conduction abnormalities:
    • late potentials
  • arrhythmias:
    • ventricular tachycardia with LBBB and frequent VES
  • family history:
    • family history of sudden death due to suspected ARVC
    • family history of ARVC on diagnostic criteria 

According to the ARVC task force modified criteria from 2010 , MRI imaging features are part of the major and minor criteria for a diagnosis of ARVC. At the time of writing (July 2016), the most widely accepted MR criteria include:

  • MRI findings compatible with a major criterion for "global or regional dysfunction and structural alterations":
    • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
    • and 1 of the following:
      • ratio of RV end-diastolic volume to BSA 110 mL/m (male) or 100 mL/m (female)
      • or RV ejection fraction <40%
  • MRI findings compatible with a minor criterion for "global or regional dysfunction and structural alterations":
    • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction and 1 of the following:
      • ratio of RV end-diastolic volume to BSA 100 to 110 mL/m (male) or 90 to 100 mL/m (female)
      • or RV ejection fraction 40% to 45%

Of note, the modified task force criteria do not include MRI detection of fat in the RV wall as a major or minor criterion, as "fatty infiltration of the myocardium by MRI has proven problematic".