Diagnostic Criteria for
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Family history
- Familial disease confirmed at necropsy or surgery
- Family history of premature sudden death (<35 years) due to suspected ARVD/C
- Family history (clinical diagnosis based on present criteria)
ECG depolarization /conduction abnormalities
- Epsilon waves or localized prolongation (³ 110 ms) of the QRS complex in the right precordial leads (V1-V3)
- Late potentials seen on signal averaged electrocardiography
ECG repolarization 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
Arrhythmias
- Sustained or nonsustained left bundle branch block type ventricular tachycardia documented on the electrocardiography, Holter monitoring or during exercise testing.
- Frequent ventricular extrasystoles (more than 1,000/24 hours on Holter monitoring).
Global and/or regional dysfunction and structural alterations *
- Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) left ventricular involvement.
- Localized 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
Tissue characteristics of walls
- Fibrofatty replacement of myocardium on endomyocardial biopsy
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*Detected by echocardiography, angiography, magnetic resonance imaging, or radionuclide scintigraphy.