Table 1 Criteria for diagnosis of ARVD [5]

   • Major

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

   • Major

   Fibrofatty replacement of myocardium on endomyocardial biopsy

III. Repolarisation Abnormalities

   • Minor

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

IV. Depolarization/Conduction Abnormalities

   • Major

   Epsilon waves or localized 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 and non-sustained) by ECG, Holter or exercise testing

   Frequent ventricular extra-systoles (>1000/24 hours) by Holter

VI. Family History

   • Major

   Familial disease confirmed at necropsy or surgery

   • Minor

   Family history of premature sudden death (<35 years) due to suspected right ventricular dysplasia

   Familial history (clinical diagnosis based on present criteria)