Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria

Criteria for RV Involvement

I. Morpho-functional ventricular abnormalities

Major: By 2D echocardiogram, CMR, or angiography:

Regional RV akinesia, dyskinesia, or bulging

Plus 1 of the following

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: By 2D echocardiogram, CMR, or angiography:

Regional RV akinesia, dyskinesia or aneurysm of RV free wall

II. Structural myocardial abnormalities

Major By CE‐CMR:

Transmural LGE (stria pattern) of ≥1 RV region(s) (inlet, outlet, and apex in 2 orthogonal views)

Major By EMB (limited indications):

Fibrous replacement of the myocardium in ≥1 sample, with or without fatty tissue

III. ECG repolarization abnormalities

Major

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 leads V1 and V2 in individuals with completed pubertal development (in the absence of complete RBBB)

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

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)

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)

V. Ventricular arrhythmias

Major

Frequent ventricular extrasystoles (>500 per 24 h), non‐sustained or sustained ventricular tachycardia of LBBB morphology*

Minor

Frequent ventricular extrasystoles (>500 per 24 h), non‐sustained or sustained ventricular tachycardia of LBBB morphology with inferior axis (“RVOT pattern”)

VI. Family history/genetics

Major

ACM confirmed in a first‐degree relative who meets diagnostic criteria

ACM confirmed pathologically at autopsy or surgery in a first‐degree relative

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

Premature sudden death (<35 y of age) due to suspected ACM in a first‐degree relative

ACM confirmed pathologically or by diagnostic criteria in second‐degree relative

Criteria for LV Involvement

I. Morpho-functional ventricular abnormalities

Minor: By 2D echocardiogram,CMR or angiography:

Global LV systolic dysfunction (depression of LV EF or reduction of echocardiographic global longitudinal strain), with or without LV dilatation (increase in LV EDV according to the imaging test specific nomograms for age, sex, and BSA)

Regional LV hypokinesia or akinesia of LV free wall, septum or both

II. Structural myocardial abnormalities

Major By CE‐CMR:

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)

III. ECG repolarization abnormalities

Minor

Inverted T waves in left precordial leads (V4–V6) without complete LBBB

IV. ECG repolarization abnormalities

Minor

Low QRS voltages (<0.5 mV peak to peak) in limb leads (in the absence of obesity, emphysema or pericardial effusion)

V. Ventricular arrhythmias

Minor

Frequent ventricular extrasystoles (>500 per 24 h) or non-sustained or sustained ventricular tachycardia with an RBBB morphology (excluding the “fascicular pattern”)

References

Graziano F, Zorzi A, Cipriani A, De Lazzari M, Bauce B, Rigato I, Brunetti G, Pilichou K, Basso C, Perazzolo Marra M, et al. The 2020 “Padua Criteria” for Diagnosis and Phenotype Characterization of Arrhythmogenic Cardiomyopathy in Clinical Practice. Journal of Clinical Medicine. 2022; 11(1):279. https://doi.org/10.3390/jcm11010279