ARVC Task Force Criteria (2010 Modified)
Definition
A multi-domain scoring system to standardize the clinical diagnosis of ARVC. No single gold standard test exists; the criteria require a combination of major and minor findings across five categories. A definitive diagnosis requires 2 major OR 1 major + 2 minor OR 4 minor criteria from different categories.
Key Concepts
Diagnostic Thresholds
- Definite ARVC: 2 major | 1 major + 2 minor | 4 minor criteria (from different categories)
- Borderline ARVC: 1 major + 1 minor | 3 minor criteria
- Possible ARVC: 1 major | 2 minor criteria
(sources/acm-hrs-2019)
Five Diagnostic Categories
1. Global/Regional Dysfunction and Structural Alterations (Echo/MRI/RV angiography)
- Echo major: Regional RV akinesia/dyskinesia/aneurysm + PLAX RVOT ≥32 mm or PSAX RVOT ≥36 mm or FAC ≤33%
- CMR major: Regional RV wall motion abnormality + RVEDV/BSA ≥110 mL/m² (male) or ≥100 mL/m² (female) or RVEF ≤40%
2. Tissue Characterization of Wall (Endomyocardial Biopsy)
- Major: Residual myocytes <60% by morphometric analysis with fibrous/fibrofatty replacement
- Minor: Residual myocytes 60–75%
3. Repolarization Abnormalities (ECG)
- Major: TWI in V1–V3 in patients >14 years without complete RBBB
- Minor: TWI in V1–V2 (no RBBB) or V4–V6; TWI in V1–V4 with complete RBBB
4. Depolarization/Conduction Abnormalities (ECG)
- Major: Epsilon wave in right precordial leads (V1–V3) — reproducible low-amplitude deflection between QRS end and T onset
- Minor: Late potentials on SAECG (≥1 of 3 parameters: fQRS ≥114 ms, LAS ≥38 ms, RMS40 ≤20 μV); Terminal activation duration ≥55 ms in V1–V3 without CRBBB
5. Arrhythmias
- Major: Non-sustained or sustained VT with LBBB and superior axis (negative/indeterminate in II, III, aVF; positive in aVL)
- Minor: LBBB VT with inferior axis or unknown axis; >500 ventricular extrasystoles/24h
6. Family History
- Major: ARVC confirmed in first-degree relative by Task Force Criteria; ARVC confirmed at autopsy/surgery; or identification of a pathogenic variant in the patient
- Minor: Unconfirmed ARVC in first-degree relative; premature SCD (<35 years) suspected ARVC; ARVC confirmed in second-degree relative
(sources/acm-hrs-2019)
Limitations
- The Task Force Criteria were designed for ARVC and have not been validated for other ACMs with overlapping phenotypes (cardiac sarcoidosis, myocarditis, ALVC).
- No analogous validated criteria exist for ALVC — an urgent unmet need. (sources/acm-hrs-2019)
- Genetic diagnosis (class 4/5 pathogenic variant) counts as a major criterion in the family history domain, contributing up to 50% toward a definitive diagnosis.
Contradictions / Open Questions
- Epsilon wave as major criterion vs. unreliable finding: The 2010 TFC treat the epsilon wave as a major diagnostic criterion (equal to RV dilatation/dysfunction or tissue pathology). ESC 2023 explicitly warns that epsilon waves have poor sensitivity/specificity and high interobserver variability, typically appearing only in severe structural disease. The 2010 TFC have not been formally updated to reflect this; clinicians who rely on epsilon waves may over- or misdiagnose ARVC. (sources/acm-hrs-2019, sources/esc-cmp-2023)
- No criteria for ALVC or biventricular disease: The Task Force Criteria were designed exclusively for RV-dominant ARVC. No validated analogous criteria exist for ALVC, and the Padua criteria (incorporating LV involvement) lack external validation. A clinically important patient group — those with biventricular or LV-dominant ACM — cannot be formally diagnosed using these criteria. (sources/acm-hrs-2019)
- Pathogenic variant as major family history criterion: A confirmed class 4/5 pathogenic variant counts as a major criterion in the family history domain, potentially contributing 50% of the score required for definitive diagnosis (1 major criterion of 2). This means a patient could receive a definitive ARVC diagnosis based partly on genetic data without any direct cardiac evaluation — a situation that may not reflect true ARVC penetrance in low-expressivity individuals. (sources/acm-hrs-2019)
Connections
- Related to concepts/Arrhythmogenic-Cardiomyopathy
- Related to entities/ARVC
- Related to entities/ALVC
- Related to concepts/Desmosome