Table 1.
Criteria for RV involvement | Criteria for LV involvement | |
---|---|---|
I. Morpho‐functional ventricular abnormalities | By 2D echocardiogram, CMR or angiography: | By 2D echocardiogram, CMR or angiography: |
Major | Minor | |
Regional RV akinesia, dyskinesia, or bulging plus one of the following: | Global LV systolic dysfunction (depression of LV EF or reduction of echocardiographic global longitudinal strain), with or without LV dilatation (increase of LV EDV according to the imaging test specific nomograms for age, sex, and BSA) | |
‐ global RV dilatation (increase of RV EDV according to the imaging test specific nomograms for age, sex and BSA) ‐ global RV systolic dysfunction (reduction of RV EF according to the imaging test specific nomograms for age and sex) | ||
Minor | Minor | |
Regional RV akinesia, dyskinesia or aneurysm of RV free wall | Regional LV hypokinesia or akinesia of LV free wall, septum, or both | |
II. Structural myocardial abnormalities | By CE‐CMR: | By CE‐CMR: |
Major | Major | |
Transmural LGE (stria pattern) of ≥1 RV region(s) (inlet, outlet, and apex in 2 orthogonal views) | LV LGE (stria pattern) of ≥1 Bull's Eye segment(s) (in 2 orthogonal views) of the free wall (subepicardial or midmyocardial), septum, or both (excluding septal junctional LGE) | |
By EMB (limited indications): | ||
Major | ||
Fibrous replacement of the myocardium in ≥1 sample, with or without fatty tissue | ||
III. ECG repolarization abnormalities | Major | Minor |
Inverted T waves in right precordial leads (V1, V2, and V3) or beyond in individuals with complete pubertal development (in the absence of complete RBBB) | Inverted T waves in left precordial leads (V4–V6) without complete LBBB | |
Minor | ||
‐ Inverted T waves in leads V1 and V2 in individuals with completed pubertal development (in the absence of complete RBBB) or ‐ Inverted T waves in V1, V2, V3 and V4 in individuals with completed pubertal development in the presence of complete RBBB | ||
IV. ECG depolarization abnormalities | Minor | Minor |
‐ Epsilon wave (reproducible low amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V1 to V3) or ‐ Terminal activation duration of QRS ≥55 ms measured from the nadir of the S wave to the end of the QRS, including R’, in V1, V2, or V3 (in the absence of complete RBBB) |
Low QRS voltages (<0.5 mV peak to peak) in limb leads (in the absence of obesity, emphysema, or pericardial effusion) | |
V. Ventricular arrhythmias | Major | Minor |
Frequent ventricular extrasystoles (>500 per 24 h), non‐sustained or sustained ventricular tachycardia of LBBB non‐inferior axis morphology | Frequent ventricular extrasystoles (>500 per 24 h), non‐sustained or sustained ventricular tachycardia with a RBBB morphology (excluding the “fascicular pattern”) | |
Minor | ||
Frequent ventricular extrasystoles (>500 per 24 h), non‐sustained or sustained ventricular tachycardia of LBBB morphology with inferior axis (“RVOT pattern”) | ||
VI. Family historygenetics | Major | |
‐ ACM confirmed in a first‐degree relative who meets diagnostic criteria or ‐ ACM confirmed pathologically at autopsy or surgery in a first‐degree relative or ‐ Identification of a pathogenic or likely pathogenetic ACM mutation in the patient under evaluation | ||
Minor | ||
‐ History of ACM in a first‐degree relative in whom it is not possible or practical to determine whether the family member meets diagnostic criteria or ‐ Premature sudden death (<35 years of age) due to suspected ACM in a first‐degree relative or ‐ ACM confirmed pathologically or by diagnostic criteria in second‐degree relative |
Note: Adapted from Corrado et al. 21
Abbreviations: ACM, arrhythmogenic cardiomyopathy; BSA, body surface area; CE‐CMR, constrast enhanced‐cardiac magnetic resonance; CMR, cardiac magnetic resonance; EDV, end diastolic volume; EF, ejection fraction; EMB, endomyocardial biopsy; LBBB, left bundle branch block; LGE, late gadolinium enhancement; LV, left ventricle; RBBB, right bundle branch block; RV, right ventricle; RVOT, right ventricular outflow tract.