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Table 1 Abbreviated Original and Revised Task Force Criteria for the diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). A Criteria I – Imaging; B Criteria V – Arrhythmias; C Criteria VI – Family History. Revised from Marcus et al.

From: Long term CMR follow up of patients with right ventricular abnormality and clinically suspected arrhythmogenic right ventricular cardiomyopathy (ARVC)

Original Task Force Criteria

Revised Task Force Criteria

A

I. Global or regional dysfunction and structural alterations; Imaging

Major

Major

-Severe dilatation and reduction of RV ejection fraction with no LV impairment

-Localized RV aneurysms

-Severe segmental dilatation of the RV

By 2D echo:

Regional RV akinesia, dyskinesia or aneurysm and 1 of the following (end diastole and corrected for body size (Parasternal long axis /BSA)):

- Parasternal long axis RVOT > 32 mm Parasternal long axis /BSA > 19 mm/m2

- Parasternal short axis RVOT > 36 mm Parasternal short axis /BSA > 21 mm/m2

-Fractional area change < 33%

By CMR:

Regional RV akinesia or dyskinesia or dyssynchrounous RV contraction and 1 of the following:

-Ratio of RV EDV to BSA > 110 ml/m2 (male) or > 100 ml/m2 (female)

-RV ejection fraction < 40%

Minor

Minor

-Mild global RV dilatation and/or ejection fraction reduction with normal LV

-Mild segmental dilatation of the RV

-Regional RV hypokinesis

By 2D echo:

Regional RV akinesia or dyskinesia and 1 of the following (end diastole and corrected for body size (Parasternal long axis /BSA))

-Parasternal long axis RVOT > 29 to < 32 mm Parasternal long axis /BSA > 16 to < 19 mm/m2

-Parasternal short axis RVOT > 32 to 36 mm Parasternal short axis /BSA > 18 to < 21 mm/m2

-RV Fractional area change > 33% to < 40%

By MRI:

Regional RV akinesia or dyskinesia or dyssynchrounous RV contraction and 1 of the following:

-Ratio of RV EDV to BSA > 100 to < 110 ml/m2 (male) or > 90 to < 100 ml/m2 (female)

-RV ejection fraction > 40% to < 45%

B

V. Arrhythmias

Major

Major

 

-Non-sustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL)

Minor

Minor

-Left bundle-branch block-type ventricular tachycardia (sustained and non-sustained)

-Frequent ventricular extra-systoles (1000 per 24 h)

-Non-sustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis

- > 500 ventricular extra-systoles per 24 h

C

VI. Family history

Major

Major

-Familial disease confirmed at necropsy or surgery

-ARVC confirmed in a first-degree relative who meets current Task Force criteria

-ARVC confirmed pathologically at autopsy or surgery in a first-degree relative

-Identification of a pathogenic mutation categorized as associated or probably associated with ARVC in the patient

Minor

Minor

-Family history of premature sudden death (35 years of age) due to suspected ARVC

-Familial history (clinical diagnosis based on present criteria)

-History of ARVC in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria

-Premature sudden death (35 years of age) due to suspected ARVC in a first-degree relative

-ARVC confirmed pathologically or by current Task Force Criteria in second-degree relative

  1. 2D, two dimensional; ARVC, arrhythmogenic right ventricular cardiomyopathy; BSA, body surface area; EDV, end-diastolic volume; LV, left ventricle; RV, right ventricle; RVOT, right ventricular outflow tract