Table 5 Diagnostic criteria for arrhythmogenic right ventricular dysplasia (ARVD)

I. Global and/or regional dysfunction and structural alterations

• Major

—Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) left and ventricular impairment

—Localized right ventricular aneurysms (akinetic or dyskinetic areas with diastolic bulging)

—Severe segmental dilatation of the right ventricle

• Minor

—Mild global right ventricular dilatation and/or ejection fraction reduction with normal left ventricle

—Mild segmental dilatation of the right ventricle

—Regional right ventricular hypokinesia

II. Tissue characterisation of walls

• Major

—Fibrofatty replacement of myocardium at endomyocardial biopsy

III. Repolarisation abnormalities

• Minor

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

IV. Depolarisation of conduction abnormalities

• Major

—Epsilon waves or localised prolongation (>110 ms) of the QRS complex in right precordial leads (V1—V3)

• Minor

—Late potentials (signal-averaged ECG)

V. Arrhythmias

• Minor

—Left bundle branch block type ventricular tachycardia (sustained or nonsustained; ECO, Holter, exercise testing)

—Frequent ventricular extrasystoles on Holter (>1,000/24 h)

VI. Family history

• Major

—Familial disease confirmed at necropsy or surgery

• Minor

—Familial history of premature sudden death (at <35 years) due to suspected right ventricular cardiomyopathy

—Familial history (clinical diagnosis based on present criteria)