Arrhythmogenic right ventricular dysplasia diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

There is no pathognomonic feature of ARVD. The diagnosis of ARVD is based on a combination of major and minor criteria. To make a diagnosis of ARVD requires either 2 major criteria or 1 major and 2 minor criteria or 4 minor criteria.

Diagnostic Criteria

Major Criteria

• Right ventricular dysfunction
  • Severe dilatation and reduction of RV ejection fraction with little or no LV impairment
  • Localized RV aneurysms
  • Severe segmental dilatation of the RV
• Tissue characterization
  • Fibrofatty replacement of myocardium on endomyocardial biopsy
• Conduction abnormalities
  • Epsilon waves in V₁ - V₃.
  • Localized prolongation (>110 ms) of QRS in V₁ - V₃
• Family history
  • Familial disease confirmed on autopsy or surgery

Minor Criteria

• Right ventricular dysfunction
  • Mild global RV dilatation and/or reduced ejection fraction with normal LV.
  • Mild segmental dilatation of the RV
  • Regional RV hypokinesis
• Tissue characterization
• Conduction abnormalities
  • Inverted T waves in V₂ and V₃ in an individual over 12 years old, in the absence of a right bundle branch block (RBBB)
  • Late potentials on signal averaged EKG.
  • Ventricular tachycardia with a left bundle branch block (LBBB) morphology
  • Frequent PVCs (> 1000 PVCs / 24 hours)
• Family history
  • Family history of sudden cardiac death before age 35
  • Family history of ARVD