Table 1 Criteria for diagnosis of ARVC/D

1. Family history

Major

Familial disease confirmed at necropsy or surgery.

Minor

Family history of premature sudden death (<35 years of age) due to suspected ARVC/D.

Family history (clinical diagnosis based on present criteria).

2. ECG depolarization/conduction abnormalities

Major

Epsilon waves or localized prolongation (>110 ms) of QRS complex in right precordial leads (V₁-V₃).

Minor

Late potentials on signal-averaged ECG.

3. ECG repolarization abnormalities

Minor

Inverted T waves in right precordial leads (V₂ and V₃) in people >12 years of age and in absence of right bundle branch block.

4. Arrhythmias

Minor

Sustained or nonsustained left bundle branch block-type ventricular tachycardia documented on ECG or Holter monitoring or during exercise testing.

Frequent ventricular extrasystoles (>1000/24 h on Holter monitoring).

5. Global or regional dysfunction and structural alterations*

Major

Severe dilatation and reduction of RV ejection fraction with no or mild LV involvement.

Localized RV aneurysms (akinetic or dyskinetic areas with diastolic bulgings). Severe segmental dilatation of RV.

Minor

Mild global RV dilatation or ejection fraction reduction with normal LV.

Mild segmental dilatation of RV.

Regional RV hypokinesia.

6. Tissue characteristics of walls

Major

Fibro-fatty replacement of myocardium on endomyocardial biopsy.