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 is not formly established by echocardiography or CT, but MRI is improving in its diagnostic capabilities by virtue of its ability to characterize the RV free wall and the anatomy of the right ventricle. 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

In 1994, the European Society of Cardiology and the International Society and Federation of Cardiology proposed diagnostic criteria for arrhythmogenic right ventricular dysplasia/cardiomyopathy. To make the diagnosis of ARVD/C, it requires either two major, one major and two minor, or four minor 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