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Table 1 Revised 2010 Task Force Criteria for ARVC*

From: Arrhythmogenic right ventricular cardiomyopathy (ARVC): cardiovascular magnetic resonance update

1. Global or regional dysfunction and structural alterations

Major

 

2D Echo 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), or

   

- PSAX RVOT ≥ 36 mm (PSAX/BSA ≥ 21 mm/m2), or

   

- Fractional area change ≤ 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 Echo Criteria

  

Regional RV akinesia or dyskinesia or dyssynchronous RV contraction AND 1 of the following measured at end diastole:

   

- PLAX RVOT ≥29 to <32 mm (PLAX/BSA ≥16 to <19 mm/m2), or

   

- PSAX RVOT ≥32 to <36 mm (PSAX/BSA ≥18 to <21 mm/m2), or

   

- Fractional area change > 33% ≤ 40%

 

CMR criteria

  

Regional RV akinesia or dyskinesia or dyssynchronous RV contraction AND 1 of the following:

   

- RV EDV/BSA ≥100 to 110 mL/m2 (male) or ≥90 to 100 mL/m2 (female)

   

- RV ejection fraction >40 to ≤ 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% 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

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 QRS ≥ 120 ms)

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 years 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 msec on standard ECG:

 

- Filtered QRS duration (fQRS) ≥ 114 msec

 

- Duration of terminal QRS < 40 microV ≥ 38 ms

 

- Root-mean-square voltage of terminal 40 ms ≤20 micro V

 

Terminal activation duration ≥ 55 ms measured from the nadir of the S-wave until the end of all depolarization deflections (including R') in V1, V2, or V3

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 of unknown axis

 

> 500 PVCs per 24 hours on Holter monitoring

6. Family History

Major

 

ARVC in first degree relative who meets Task Force Criteria

 

ARVC confirmed pathologically at autopsy or surgery in first degree relative

 

Identification of pathogenic mutation categorized as associated or probably associated with ARVC in the patient under evaluation

Minor

 

History of ARVC in first degree relative in whom it is not possible to determine whether the family member meets 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