Diagnostic criteria would be fulfilled by the presence of:

• 2 major criteria or
• 1 major plus 2 minor criteria or
• 4 minor criteria

The criteria must come from different categories (I-VI).

I. Global and/or Regional Dysfunction and Structural Alterations*

MAJOR:


• Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV 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 Walls

MAJOR:


• Fibrofatty replacement of myocardium on endomyocardial biopsy.

III. Repolarisation Abnormalities

MINOR:


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

IV. Depolarisation/Conduction Abnormalities

MAJOR:


• Epsilon waves or localised prolongation (>110ms) 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 nonsustained) ECG, Holter, exercise testing.
Frequent ventricular extrasystoles (>1000/24 hours) (Holter.)

VI. Family History

MAJOR:


• Familial disease confirmed at necropsy or surgery.

MINOR:


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

• Familial history (clinical diagnosis based on present criteria.)

* Detected by echocardiography, angiography, magnetic imaging, or radionuclide scinitigraphy. ECG=electrocardiogram; LV =left ventricle.

McKenna WJ, Thiene G, Nava A, Fontaliran F. Blomstrom-Lundqvist C, Fontaine G, Camerini on behalf of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology, supported by the Schoepfer Association. Diagnosis of Arrhythmogenic Right Ventricular Dysplasia/cardiomyopathy. Br Heart J 1994;71:215-218.


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