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2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC)

2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC)
  Revised Task Force criteria
I. Global or regional dysfunction and structural alterations*
Major By 2D echo:
  • Regional RV akinesia, dyskinesia, or aneurysm
  • and 1 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 1 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 By 2D echo:
  • Regional RV akinesia or dyskinesia
  • and 1 of the following (end diastole):
    • PLAX RVOT ≥29 to <32 mm (corrected for body size [PLAX/BSA] ≥16 to <19 mm/m2)
    • PSAX RVOT ≥32 to <36 mm (corrected for body size [PSAX/BSA] ≥18 to <21 mm/m2)
    • or fractional area change >33% to ≤40%
By MRI:
  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • and 1 of the following:
    • Ratio of RV end-diastolic volume to BSA ≥100 to <110 mL/m2 (male) or ≥90 to <100 mL/m2 (female)
    • or RV ejection fraction >40% to ≤45%
II. Tissue characterization of wall
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
  • Residual myocytes 60% to 75% by morphometric analysis (or 50% to 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. Repolarization abnormalities
Major
  • 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 ms)
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
Major
  • 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
  • Late potentials by SAECG in ≥1 of the following 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. Arrhythmias
Major
  • Nonsustained 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
  • Nonsustained 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 extrasystoles per 24 hours (Holter)
VI. Family history
Major
  • 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 under evaluation
Minor
  • 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
Diagnostic terminology for revised criteria:
  • Definite diagnosis: 2 major, OR 1 major and 2 minor criteria, OR 4 minor from different categories
  • Borderline diagnosis: 1 major and 1 minor, OR 3 minor criteria from different categories
  • Possible diagnosis: 1 major, OR 2 minor criteria from different categories
ARVC: arrhythmogenic right ventricular cardiomyopathy; 2D: 2-dimensional; echo: echocardiography; PLAX: parasternal long-axis view; RVOT: RV outflow tract; BSA: body surface area; PSAX: parasternal short-axis view; MRI: magnetic resonance imaging; SAECG: signal-averaged electrocardiogram; aVF: augmented voltage unipolar left foot lead; aVL: augmented voltage unipolar left arm lead.
* 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 that alters or is expected to alter the encoded protein, is unobserved or rare in a large non-ARVC 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.
Modified with permission from: Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force criteria. Circulation 2010; 121:1533. Copyright © 2010 Lippincott Williams & Wilkins.
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