Clinical Resources
ARVC/D Diagnostic Criteria
2010 Revised Task Force Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy
Check all criteria present. Each category contributes Major (2 pts) or Minor (1 pt) criteria toward the diagnosis.
I
Global or Regional Dysfunction & Structural Alterations
0 Maj
0 Min
Echo — Regional RV akinesia/dyskinesia/aneurysm + one of:
PLAX RVOT ≥32 mm (≥19 mm/m²) · PSAX RVOT ≥36 mm (≥21 mm/m²) · FAC ≤33%
MRI — Regional RV akinesia/dyskinesia or dyssynchronous RV contraction + one of:
RV EDV/BSA ≥110 mL/m² (male) or ≥100 mL/m² (female) · RVEF ≤40%
RV angiography — Regional RV akinesia/dyskinesia/aneurysm
Echo — Regional RV akinesia/dyskinesia + one of:
PLAX RVOT 29–31 mm (16–18 mm/m²) · PSAX RVOT 32–35 mm (18–20 mm/m²) · FAC 33–40%
MRI — Regional RV akinesia/dyskinesia or dyssynchronous RV contraction + one of:
RV EDV/BSA 100–109 mL/m² (male) or 90–99 mL/m² (female) · RVEF 40–45%
II
Tissue Characterization of Wall
0 Maj
0 Min
Residual myocytes <60% by morphometric analysis (or <50% if estimated) with fibrous replacement of the RV free wall myocardium ± fatty replacement on endomyocardial biopsy
Residual myocytes 60–75% by morphometric analysis (or 50–65% if estimated) with fibrous replacement of the RV free wall myocardium ± fatty replacement on endomyocardial biopsy
III
Repolarization Abnormalities
0 Maj
0 Min
Inverted T waves in right precordial leads (V1, V2, V3) or beyond — in absence of complete RBBB (QRS ≥120 ms)
Age >14 years
Inverted T waves in leads V1 and V2 in absence of complete RBBB, or in V4, V5, V6
Age >14 years
Inverted T waves in leads V1, V2, V3, V4 in presence of complete RBBB
IV
Depolarization / Conduction Abnormalities
0 Maj
0 Min
Epsilon wave — reproducible low-amplitude signal between end of QRS and onset of T wave in right precordial leads (V1–V3)
Late potentials on signal-averaged ECG (SAECG) — ≥1 of 3 parameters present in absence of QRS ≥110 ms:
Filtered QRS ≥114 ms · LAS (duration of terminal QRS <40 µV) ≥38 ms · RMS40 (RMS voltage of terminal 40 ms) ≤20 µV
Terminal activation duration of QRS ≥55 ms — measured from nadir of S wave to end of QRS, including R', in V1, V2, or V3 — in absence of complete RBBB
V
Arrhythmias
0 Maj
0 Min
Non-sustained or sustained VT of LBBB morphology with superior QRS axis
Negative or indeterminate QRS in leads II, III, aVF and positive in lead aVL
Non-sustained or sustained VT of RVOT configuration — LBBB morphology with inferior axis or of unknown axis
>500 PVCs/24 hours on Holter — with LBBB morphology
VI
Family History & Genetics
0 Maj
0 Min
ARVC/D confirmed in first-degree relative meeting Task Force Criteria
ARVC/D confirmed at autopsy or surgery in first-degree relative
Identification of a pathogenic mutation (desmosomal gene) categorized as associated with or probably associated with ARVC/D
PKP2, DSP, DSC2, DSG2, JUP, TMEM43, or other
History of ARVC/D in first-degree relative in whom it is not confirmed meeting current Task Force Criteria
Premature sudden death (<35 years) due to suspected ARVC/D in a first-degree relative
ARVC/D confirmed at autopsy or surgery in second-degree relative
Major
0
Minor
0
Equivalent
0
Diagnostic Thresholds (2010 Task Force)
| Classification | Criteria Required |
|---|---|
| Definite | 2 Major · OR · 1 Major + 2 Minor · OR · 4 Minor (from different categories) |
| Borderline | 1 Major + 1 Minor · OR · 3 Minor (from different categories) |
| Possible | 1 Major · OR · 2 Minor (from different categories) |
Criteria must come from different categories for multi-minor rules. "Category-equivalent" score shown = (2 × Major) + (1 × Minor) for reference only; formal diagnosis requires reading the category-combination rules above. Criteria apply to index patients; cascade screening in relatives uses modified thresholds.