McKenna et al listed criteria for the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. The authors are from multiple hospitals from around the world participating in the European Society of Cardiology and the International Society and Federation of Cardiology.


Major criteria:

(1) severe dilatation and reduction of the right ventricular ejection fraction with no or only mild left ventricular impairment

(2) localized aneurysms of the right ventricle (due to akinetic or dyskinetic areas bulging during diastole)

(3) severe segmental dilatation of the right ventricle

(4) fibrofatty replacement of myocardium on endomyocardial biopsy

(5) episolon waves or localized prolongation of the QRS complex in V1 to V3 (> 100 milliseconds in the right precordial leads)

(6) familial disease confirmed at surgery or autopsy


Minor criteria:

(1) (mild global right ventricular dilatation and/or reduction of ejection fraction) AND (normal left ventricle)

(2) mild segmental dilatation of the right ventricle

(3) inverted T waves in V2 and V3 in someone > 12 years old without right bundle branch block

(4) late potentials in signal averaged ECG

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

(6) family history based on clinical diagnosis


The diagnosis of right ventricular dysplasia/cardiomyopathy can be made by one or more of the following:

(1) >= 2 major criteria

(2) 1 major criteria AND >= 2 minor criteria

(3) >= 4 minor criteria


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