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 |