Criteria for diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC)6
| I. Family history |
| Major |
| Familial disease confirmed at necropsy or surgery |
| Minor |
| Family history of premature sudden death (< 35 years) caused by suspected ARVC |
| Family history (clinical diagnosis based on present criteria) |
| II. ECG depolarisation/conduction abnormalities |
| Major |
| Epsilon waves or localised prolongation (⩾ 110 ms) of the QRS complex in the right precordial leads (V1–V3) |
| Minor |
| Late potentials seen on signal averaged ECG |
| III. ECG repolarisation abnormalities |
| Minor |
| Inverted T waves in right precordial leads (V2 and V3) in people >12 years and in the absence of right bundle branch block |
| IV. Arrhythmias |
| Minor |
| Sustained or non-sustained left bundle branch block type ventricular tachycardia documented on the ECG, Holter monitoring or during exercise testing |
| Frequent ventricular extrasystoles (more than 1000/24 hours on Holter monitoring) |
| V. Global and/or regional dysfunction and structural alterations 1-150 |
| Major |
| Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) left ventricular involvement |
| Localised right ventricular aneurysms (akinetic or dyskinetic areas with diastolic bulgings) |
| 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 |
| VI. Tissue characteristics of walls |
| Major |
| Fibrofatty replacement of myocardium on endomyocardial biopsy |
↵1-150 Detected by echocardiography, angiography, magnetic resonance imaging, or radionuclide scintigraphy.