Arrhythmogenic right ventricular cardiomyopathy diagnostic criteria

For the diagnosis of arrthymogenic right ventricular dysplasia 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
  • prolongued 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 ARVD
  • family history of ARVD on diagnostic criteria 

According to the ARVD task force modified criteria from 2010 ³, MRI imaging features are part of the major and minor criteria for a diagnosis of ARVD.

• 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
    • ratio of RV end-diastolic volume to BSA 110 mL/m2 (male) or 100 mL/m2 (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
    • ratio of RV end-diastolic volume to BSA 100 to 110 mL/m2 (male) or 90 to 100 mL/m2 (female)—
    • or RV ejection fraction 40% to 45%

Of note, the modified task force does 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"."