TABLE 1.

Revised 2010 TFC for ARVD/C^*

1 Global or Regional Dysfunction and Structural Alterations

Major

2D echocardiographic criteria

Regional RV akinesia, dyskinesia, or aneurysm and 1 of the following measured at end diastole: PLAX RVOT ≥32 mm (PLAX/BSA ≥19 mm/m2), PSAX RVOT ≥36 mm (PSAX/BSA ≥21 mm/m2), or FAC ≤33%

CMR criteria

Regional RV akinesia or dyskinesia or dyssynchronous RV contraction and 1 of the following: RV EDV/BSA ≥110 ml/m2 (male) or ≥100 ml/m2 (female) RV ejection fraction ≤40%

RV angiography criteria

Regional RV akinesia, dyskinesia, or aneurysm

Minor

2D echocardiographic criteria

Regional RV akinesia or dyskinesia and 1 of the following measured at end diastole: PLAX RVOT ≥29 to <32 mm (PLAX/BSA ≥16–<19 mm/m2), or PSAX RVOT ≥32 to <36 mm (PSAX/BSA ≥18–<21 mm/m2), or FAC >33% ≤40%

CMR criteria

Regional RV akinesia or dyskinesia or dyssynchronous RV contraction and 1 of the following: RV EDV/BSA ≥100–<110 ml/m2 (male) or ≥90–<100 ml/m2 (female) RV ejection fraction >40–≤45%

2 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%–75% by morphometric analysis (or 50%–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

3 Repolarization Abnormalities

Major

Inverted T waves in right precordial leads (V1, V2, and V3) or beyond in individuals >14 yrs of age (in the absence of complete RBBB)

Minor

Inverted T waves in V1 and V2 in individuals >14 yrs of age (in the absence of complete RBBB) or in V4, V5, and V6

Inverted T waves in leads V1, V2, V3, and V4 in individuals >14 yrs of age in the presence of a complete RBBB

4 Depolarization/Conduction Abnormalities

Major

Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of T-wave) in the right precordial leads (V1–V3)

Minor

Late potentials by SAECG in ≥1 of 3 parameters in the absence of a QRSd of ≥110 ms on standard ECG: Filtered QRS duration (≥114 ms) Duration of terminal QRS <40 μV ≥38 ms Root mean square voltage of terminal 40 ms ≤20 μV

Terminal activation duration of ≥55 ms measured from the nadir of the S-wave until the end of all depolarization deflections in the absence of complete RBBB

5 Arrhythmias

Major

Nonsustained or sustained VT of LBBB morphology with superior axis

Minor

Nonsustained or sustained VT of RVOT configuration, LBBB morphology with inferior axis or with unknown axis

>500 PVCs per 24 h on Holter monitoring

6 Family History

Major

ARVD/C in first-degree relative who meets current TFC

ARVD/C confirmed pathologically at autopsy or surgery in a first-degree relative

Identification of a pathogenic mutation categorized as associated or probably associated with ARVD/C in the patient under evaluation

Minor

History of ARVD/C in first-degree relative in whom it is not possible to determine whether the family member meets current TFC

Premature sudden death (<35 yrs of age) due to suspected ARVD/C in a first-degree relative

ARVD/C confirmed pathologically or by current TFC in second-degree relative

Major criteria count as 2 TFC points, and minor criteria count as 1 TFC point. Definite ARVD/C diagnosis is made with ≥4 TFC points, borderline ARVD/C diagnosis is made with 3 TFC points, and possible ARVD/C diagnosis is made with 2 TFC points.

^*Adapted from Marcus et al. (6).

ARVD/C = arrhythmogenic right ventricular dysplasia/cardiomyopathy; BSA = body surface area; CMR = cardiac magnetic resonance; ECG = electrocardiogram; EDV = end-diastolic volume; FAC = fractional area change; LBBB = left bundle branch block; PLAX = parasternal long axis; PSAX = parasternal short axis; PVC = premature ventricular complex; RBBB = right bundle branch block; RV = right ventricular; RVOT = right ventricular outflow tract; SAECG = signal-averaged electrocardiogram; TFC = Task Force Criteria; 2D = 2-dimensional; VT = ventricular tachycardia.