Evidence Based Clinical Support CARDIAC MRI

** Proposed diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy from the Task Force of the Working Group on 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.*

 Family history

** Familial disease confirmed at necropsy or surgery (Major criterion)

** Family history of premature sudden death (<35 years old) caused by suspected ARVD (Minor criterion)

** Family history (clinical diagnosis based on present criteria) (Minor criterion)

o ECG depolarization/conduction abnormalities

** Epsilon waves or localized prolongation (=110 ms) of the QRS complex in the right precordial leads (V1-V3) (Major criterion)

** Late potentials seen on signal averaged ECG (Minor criterion)

o ECG repolarization abnormalities

** Inverted T waves in right precordial leads (V2 and V3) in patients aged  >12 y and in the absence of right bundle branch block (Minor criterion)

o Arrhythmias

** Sustained or nonsustained left bundle branch block type ventricular tachycardia documented  on ECG or Holter monitoring, or during exercise testing (Minor criterion)

** Frequent ventricular extrasystoles (>1000/24 h on Holter monitoring) (Minor criterion)

o Global or regional dysfunction and structural alterations

** Severe dilatation and reduction of RV ejection fraction with no (or only mild) LV involvement (Major criterion)

** Localized RV aneurysms (akinetic or dyskinetic areas with diastolic bulging) (Major criterion)

** Severe segmental dilatation of the right ventricle (Major criterion)

** Mild global RV dilatation or ejection fraction reduction with normal left ventricle (Minor criterion)

** Mild segmental dilatation of the right ventricle (Minor criterion)

** Regional RV hypokinesia (Minor criterion)

o Tissue characteristics of walls

** Fibrofatty replacement of myocardium on endomyocardial biopsy (Major criterion)

o The diagnosis of ARVD/ARVC requires the presence of 2 major criteria or 1 major plus 2 minor or 4 minor criteria.