| 1. Global or regional dysfunction and structural alterations |
| Major |
At the two-dimensional echocardiogram: |
| - Regional right ventricular akinesis, dyskinesis or aneurysm |
| And one of the following (end-diastolic): |
| - RVOT PLAX ≥ 32 mm (corrected for the body surface area − PLAX/BSA ≥ 19 mm/m2) |
| - RVOT PSAX ≥ 36 mm (corrected for the body surface area − PLAX/BSA ≥ 21 mm/m2) |
| - Or fractional alteration of the area ≤ 33% |
| At the MRI: |
| - Regional right ventricular akinesis, dyskinesis or dyssynchrony in right ventricular contractions |
| And one of the following: |
| Ratio of right ventricular end-diastolic volume and BSA ≥ 110 mL/m2 (male) or BSA ≥ 100 mL / m2 (female) |
| -- Or right ventricular ejection fraction ≤ 40% |
| At the right ventricular angiography: |
| - Right ventricular regional akinesis, dyskinesis or aneurysm |
| Minor |
At the two-dimensional echocardiogram: |
| - Right ventricular regional akinesis or dyskinesis |
| And one of the following (end-diastolic): |
| - RVOT PLAX ≥ 29 mm and < 32 mm (corrected for the body surface area − PLAX/BSA ≥ 16 mm/m2 and < 19 mm/m2) |
| - RVOT PSAX ≥ 32 mm and < 36 mm (corrected for the body surface area − PLAX/BSA ≥ 18 mm/m2 and < 21 mm/m2) |
| - Or fractional alteration of the area > 33% and ≤ 40% |
| At the MRI: |
| - Regional right ventricular akinesis or dyskinesis or dyssynchrony in right ventricular contraction |
| And one of the following: |
| - Ratio of right ventricular end-diastolic volume and BSA ≥ 100 and < 110 mL/m2 (man) or ≥ 90 and < 100 mL/m2 (woman) |
| - Or |
| Right ventricular ejection fraction > 40% and ≤ 45% |
|
2. Histological characterization of the ventricular wall
|
| Major |
- Residual myocytes <60% in the morphometric analysis (or <50% by estimation) with fibrous replacement of the right ventricular free wall in more than one sample, with or without adipose replacement in myocardial biopsy |
| Minor |
- 60-75% of residual myocytes in the morphometric analysis (or 50-65% by estimation) with fibrous replacement of right ventricular free wall in more than one sample, with or without adipose replacement in myocardial biopsy |
|
3. Ventricular repolarization alterations
|
| Major |
- Inverted T waves in V1, V2 or V3 in individuals aged > 14 years in the absence of complete RBBB with QRS ≥ 120 ms |
| Minor |
- Inverted T waves in V1 and V2 in individuals aged > 14 years in the absence of complete RBBB with QRS ≥ 120 ms or in V4, V5 or V6 |
| Inverted T waves in V1, V2, V3 and V4 in subjects aged > 14 years in the presence of complete RBBB with QRS ≥ 120 ms |
|
4. Conduction/depolarization alterations
|
| Major |
- Epsilon wave in leads V1 to V3 |
| Minor |
- Duration of QRS terminal activation ≥ 55 ms measured from the S wave nadir to the end of QRS, including R’ at V1, V2, V3 in the absence of complete RBBB of the His bundle |
| - High-resolution ECG late potentials in more than one of the following three parameters in the absence of QRS ≥ 110 ms on the standard 12-lead ECG: |
| - Duration of filtered QRS (fQRS) ≥ 114 ms |
| - QRS terminal duration < 40 µV (low amplitude signal duration) ≥ 38 ms |
| - Root mean square of the potential in the 40 ms terminals of ventricular activation (MRIS40 - mV) ≤ 20 µV |
|
5. Arrythmias
|
| Major |
- Sustained or non-sustained ventricular tachycardia with complete LBBB morphology with superior axis (QRS negative or undetermined in II, III, aVF and positive in aVL) |
| Minor |
- Sustained or non-sustained ventricular tachycardia with right ventricular outflow tract configuration, complete LBBB morphology with inferior axis (QRS positive in II, III and aVF and negative in aVL) or of indeterminate axis |
| - > 500 ventricular extrasystoles in the 24-hr Holter monitoring |
|
6. Family history
|
| Major |
- Confirmed ARVD in a first-degree relative meeting the Task Force criteria |
| - ARVD confirmed by histopathology at the autopsy or surgery in first-degree relative |
| - Identification of pathogenic mutation categorized as associated or likely to be associated with ARVD in a patient undergoing evaluation |
| Minor |
- History of ARVD in a first-degree relative in whom it is not possible or the feasibility of confirming the presence of Task Force criteria is difficult |
| - Sudden cardiac death (age < 35 years) due to suspected ARVD in first-degree relative |
| - ARVD confirmed by histopathology or according to the current Task Force criteria in second-degree relative |
