Table 2

Original and revised task force criteria for ARVC/D diagnosis

Original task force criteriaRevised task force criteria
I. Global or regional dysfunction and structural alterations*I. Global or regional dysfunction and structural alterations*

  • Major

    • Severe dilation and reduction of RV ejection fraction with no (or only mild) LV impairment

    • Localised RV aneurysms (akinetic or dyskinetic areas with diastolic bulging)

    • Severe segmental dilation of the RV

  • Major

    • By 2-D echo:

      • Regional RV akinesia, dyskinesia or aneurysm

      • and one of the following (end diastole):

        • — PLAX RVOT ≥32 mm (corrected for body size (PLAX/BSA) ≥19 mm/m2)

        • — PSAX RVOT ≥36 mm (corrected for body size (PSAX/BSA) ≥21 mm/m2)

        • — or fractional area change ≤33%

    • By MRI:

      • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction

      • and one of the following:

        • — Ratio of RV end-diastolic volume to BSA ≥110 ml/m2 (male) or ≥100 ml/m2 (female)

        • — or RV ejection fraction ≤40%

    • By RV angiography:

      • Regional RV akinesia, dyskinesia or aneurysm

  • Minor

    • Mild global RV dilation and/or ejection fraction reduction with normal LV

    • Mild segmental dilation of the RV

    • Regional RV hypokinesia

  • Minor

    • By 2-D echo:

      • Regional RV akinesia or dyskinesia

      • and one of the following (end diastole):

        • — PLAX RVOT ≥29–<32 mm (corrected for body size (PLAX/BSA) ≥16–<19 mm/m2)

        • — PSAX RVOT ≥32–<36 mm (corrected for body size (PSAX/BSA) ≥18–<21 mm/m2)

        • — or fractional area change >33–≤40%

    • By MRI:

      • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction

      • and one of the following:

        • — Ratio of RV end-diastolic volume to BSA ≥100–<110 ml/m2 (male) or ≥90–<100 ml/m2 (female)

        • — or RV ejection fraction >40–≤45%

II. Tissue characterisation of wallII. Tissue characterisation of wall

  • Major

    • Fibrofatty replacement of myocardium on endomyocardial biopsy

  • Major

    • Residual myocytes <60% by morphometric analysis (or <50% if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy

Minor

  • Minor

    • Residual myocytes 60–75% by morphometric analysis (or 50–65% if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy

III. Repolarisation abnormalitiesIII. Repolarisation abnormalities
Major

  • Major

    • Inverted T waves in right praecordial leads (V1, V2 and V3) or beyond in individuals >14 years of age (in the absence of complete RBBB QRS ≥120 ms)

  • Minor

    • Inverted T waves in right praecordial leads (V2 and V3) (individuals aged >12 years, in absence of RBBB)

  • Minor

    • Inverted T waves in leads V1 and V2 in individuals >14 years of age (in the absence of complete RBBB) or in V4, V5, or V6

    • Inverted T waves in leads V1, V2, V3 and V4 in individuals >14 years of age in the presence of complete RBBB

IV. Depolarisation/conduction abnormalitiesIV. Depolarisation/conduction abnormalities

  • Major

    • Epsilon waves or localised prolongation (>110 ms) of the QRS complex in right praecordial leads (V1–V3)

  • Major

    • Epsilon wave (reproducible low amplitude signals between end of QRS complex to onset of the T wave) in the right praecordial leads (V1–V3)

  • Minor

    • Late potentials (SAECG)

  • Minor

    • Late potentials by SAECG in ≥1 of 3 parameters in the absence of a QRS duration of ≥110 ms on the standard ECG

    • Filtered QRS duration (fQRS) ≥114 ms

    • Duration of terminal QRS <40 μV (low amplitude signal duration) ≥38 ms

    • Root-mean-square voltage of terminal 40 ms ≤20 μV

    • Terminal activation duration of QRS ≥55 ms measured from the nadir of the S wave to the end of the QRS, including R', in V1, V2 or V3, in the absence of complete right bundle branch block

V. ArrhythmiasV. Arrhythmias
Major

  • Major

    • Non-sustained or sustained VT of LBBB morphology with superior axis (negative or indeterminate QRS in leads II, III and aVF and positive in lead aVL)

  • Minor

    • LBBB-type VT (sustained and non-sustained) (ECG, Holter, exercise)

    • Frequent ventricular extrasystoles (>1000 per 24 h) (Holter)

  • Minor

    • Non-sustained or sustained VT of RV outflow configuration, LBBB morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis

    • >500 ventricular extrasystoles per 24 h (Holter)

VI. Family historyVI. Family history

  • Major

    • Familial disease confirmed at necropsy or surgery

  • Major

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

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

    • Identification of a pathogenic mutation† categorised as associated or probably associated with ARVC/D in the patient under evaluation

  • Modified from Marcus et al.12

  • ↵* Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria.

  • ↵† A pathogenic mutation is a DNA alteration associated with ARVC/D that alters or is expected to alter the encoded protein, is unobserved or rare in a large non-ARVC/D control population, and either alters or is predicted to alter the structure or function of the protein or has demonstrated linkage to the disease phenotype in a conclusive pedigree.

  • ARVC/D, arrhythmogenic right ventricular cardiomyopathy/dysplasia; BSA, body surface area; LBBB, left bundle branch block; LV, left ventricle; PLAX, parasternal long-axis view; PSAX, parasternal short-axis view; RBBB, right bundle branch block; RV, right ventricle; RVOT, right ventricle outflow tract; SAECG, signal-averaged ECG; VT, ventricular tachycardia.