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 six categories. A definitive diagnosis requires 2 major OR 1 major + 2 minor OR 4 minor criteria from different categories.
This page describes the 2010 revised International Task Force criteria, which remain the version cited by the 2023 ESC cardiomyopathy guidelines but have been superseded in specialist ACM practice by the 2020 Padua criteria and the 2023 European Task Force criteria. The evolution is summarised on concepts/ACM-Diagnostic-Criteria. (sources/acm-evolving-criteria-jaha-2021, rating: high; sources/acm-european-tf-criteria-rcm-2024, rating: high)
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 existed for ALVC in 2010 — the gap Padua and the 2023 European TF criteria were written to fill. (sources/acm-padua-criteria-ijc-2020, rating: high)
- 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.
What the 2020 and 2023 Criteria Changed
- CMR tissue characterisation replaced the biopsy-only structural domain. RV LGE and LV LGE became structural criteria, and LV LGE is the major criterion for left-sided disease. (sources/acm-padua-criteria-ijc-2020)
- LV criteria were added: global LV systolic dysfunction and regional hypokinesia/akinesia as minor morpho-functional findings; lateral T-wave inversion and low limb-lead voltages as ECG findings; RBBB-morphology arrhythmia as an arrhythmic finding.
- Epsilon wave downgraded from major to minor; signal-averaged ECG removed.
- The severity-based major/minor split for RV dilatation/dysfunction was abolished; regional wall-motion abnormality alone became a new minor criterion.
- ALVC diagnosis: Padua required a pathogenic ACM variant; the 2023 European TF criteria removed that requirement and allow clinical diagnosis regardless of genotype or aetiology. (sources/acm-european-tf-criteria-rcm-2024)
- A morpho-functional or structural criterion is mandatory in Padua/2023, because genetic, ECG and arrhythmic findings alone cannot diagnose a structural cardiomyopathy. (sources/acm-padua-criteria-ijc-2020)
- Diagnostic yield: applying Padua to 112 patients reclassified 51 of 87 definite ARVC as biventricular and 7 of 9 possible cases as ALVC. (sources/acm-evolving-criteria-jaha-2021)
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 2010 Task Force Criteria were designed exclusively for RV-dominant ARVC, so a clinically important group — biventricular and LV-dominant ACM — cannot be formally diagnosed with them. Padua (2020) and the European TF (2023) now provide LV criteria, but both lack prospective validation, so the historical gap has been filled with an unvalidated instrument rather than closed. (sources/acm-hrs-2019; sources/acm-padua-criteria-ijc-2020; sources/acm-european-tf-criteria-rcm-2024)
- 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 concepts/ACM-Diagnostic-Criteria — the 1994→2010→2020→2023 evolution
- Related to entities/ARVC
- Related to entities/ALVC
- Related to entities/NDLVC
- Related to concepts/Desmosome
- Related to concepts/Late-Gadolinium-Enhancement