Table 1

Criteria for diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC)6

I. Family history
 Familial disease confirmed at necropsy or surgery
 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
 Epsilon waves or localised prolongation (⩾ 110 ms) of the QRS complex in the right precordial leads (V1–V3)
 Late potentials seen on signal averaged ECG
III. ECG repolarisation abnormalities
 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
 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
 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
 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
 Fibrofatty replacement of myocardium on endomyocardial biopsy
  • 1-150 Detected by echocardiography, angiography, magnetic resonance imaging, or radionuclide scintigraphy.