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. Author manuscript; available in PMC: 2011 Apr 6.
Published in final edited form as: Circulation. 2010 Feb 19;121(13):1533–1541. doi: 10.1161/CIRCULATIONAHA.108.840827

Table 1.

ORIGINAL TASK FORCE CRITERIA REVISED TASK FORCE CRITERIA
I. Global and/or Regional Dysfunction and Structural Alterations I. Global and/or Regional Dysfunction and Structural Alterations*
  • Major

  • Major (by 2D echo)

Regional RV akinesia, dyskinesia or aneurysm.
Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV impairment. And one of the following (end diastole):
Parasternal long axis view RVOT (PLAX) ≥ 32 mm
Corrected for body size (PLAX/BSA) ≥ 19 mm/m2
Localized right ventricular aneurysms (akinetic or dyskinetic areas with diastolic bulging). Parasternal short axis view RVOT (PSAX) ≥ 36 mm
Corrected for body size(PSAX/BSA) ≥ 21 mm/m2
or
Severe segmental dilatation of the right ventricle.
Fractional area change (FAC) ≤ 33%
  • Major (by MRI)

Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
And one of the following:
Right ventricular end diastolic volume (RVEDV/BSA) ≥110 ml/m2 male
≥100 ml/m2 female
OR
Right ventricular ejection fraction (RVEF) ≤40%
  • Major (by RV angiography)

Regional RV akinesia, dyskinesia or aneurysm
  • Minor

  • Minor (by 2D echo)

Regional RV akinesia or dyskinesia
Mild global right ventricular dilatation and/or ejection fraction reduction with normal left ventricle. And one of the following (end diastole):
Parasternal long axis view RVOT (PLAX) ≥ 29 - < 32 mm
Mild segmental dilatation of the right ventricle. Corrected for body size (PLAX/BSA) ≥ 16 - < 19 mm/m2i
Regional right ventricular hypokinesia. Parasternal short axis view RVOT (PSAX) ≥ 32 - < 36 mm
Corrected for body size (PSAX/BSA) ≥ 18 - < 21 mm/m2
or
Fractional area change (FAC) > 33% - ≤ 40%
  • Minor (by MRI)

Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
And one of the following:
Right ventricular end diastolic volume/BSA ≥100 - < 110 ml/m2 male
≥90 - < 100 ml/m2 female
OR
Right ventricular ejection fraction (RVEF) > 40% - ≤ 45%
II. Tissue Characterization of Wall II. Tissue Characterization of Wall
  • Major

  • Major

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

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

  • Major

Inverted T waves in right precordial leads (V2 and V3) (people aged >12 years, in absence of right bundle branch block). Inverted T waves in right precordial leads (V1, V2 and V3) or beyond in individuals > 14 years of age (in the absence of complete right bundle branch block QRS ≥ 120 msecs).
  • Minor

Inverted T waves in leads V1 and V2 in individuals > 14 years of age (in the absence of complete right bundle branch block), 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 right bundle branch block.
IV. Depolarization/Conduction Abnormalities IV. Depolarization/Conduction Abnormalities
  • Major

  • Major

Epsilon waves or localized prolongation (>110 ms) of the QRS complex in right precordial leads (V1 – V3). Epsilon wave (reproducible low amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V1 to V3)
  • Minor

  • Minor

Late potentials (signal-averaged ECG). Late potentials by signal averaged ECG in at least one of three parameters in the absence of a QRS duration of ≥110 msecs on the standard ECG.
Filtered QRS duration (fQRS) ≥114 msecs
Duration of terminal QRS < 40 μV (LAS) ≥38 msecs
RMS voltage of terminal 40 msecs ≥20 μV
Terminal activation duration of QRS ≥ 55ms 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. Arrhythmias V. Arrhythmias
  • Minor

  • Major

Left bundle branch block type ventricular tachycardia (sustained and nonsustained) (ECG, Holter, exercise testing). Non-sustained or sustained VT of left bundle branch morphology with superior axis (negative or indeterminate QRS in II, III, AVF and positive in AVL)
  • Minor

Non sustained or sustained VT of right ventricular outflow configuration, LBBB morphology with inferior axis (positive QRS in II, III, AVF and negative in AVL) or of unknown axis.
Frequent ventricular extrasystoles (>1000/24 hours) (Holter). Greater than 500 ventricular extrasystoles/24 hours by Holter
VI. Family History VI. Family History
  • Major

  • Major

Familial disease confirmed at necropsy or surgery. 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 categorized as associated or probably associated with ARVC/D in the patient under evaluation.
  • Minor

  • Minor

Family history of premature sudden death (<35 years) due to suspected right ventricular dysplasia. History of ARVC/D in a first degree relative in whom it is not possible or practical to determine if the family member meets current task force criteria.
Familial history (clinical diagnosis based on present criteria). Premature sudden death (<35 years) due to suspected ARVC/D in a first degree relative.
ARVC/D confirmed pathologically or by current Task Force Criteria in second degree relative.
*

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.

Diagnostic terminology for original criteria Diagnostic terminology for revised criteria
This diagnosis is fulfilled by the presence of two major, or one major plus two minor criteria or four minor criteria from different groups
  • Definite diagnosis: 2 major or 1 major and 2 minor criteria or 4 minor from different categories
  • Borderline: 1 major and 1 minor or 3 minor criteria from different categories
  • Possible: 1 major or 2 minor criteria from different categories