| 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%
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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%
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By RV angiography: - Regional RV akinesia, dyskinesia, or aneurysm
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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%
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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
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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)
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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
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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)
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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
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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)
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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)
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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
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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
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