Table 1.
Category | Diagnostic Criteria |
---|---|
Morpho-functional ventricular abnormalities | Minor: Global LV systolic dysfunction with or without LV dilation (assessed by echocardiography, CMR, or angiography) Minor: Regional LV hypokinesia or akinesia of the LV free wall, septum, or both |
Structural myocardial abnormalities | Major: LV LGE (stria pattern) of ≥1 Bull’s Eye segment(s) (in two orthogonal views) of the free wall (subepicardial or midmyocardial), septum, or both (excluding septal junctional LGE) |
Repolarization abnormalities | Minor: Inverted T waves in left precordial leads (V4–V6), in the absence of complete left bundle branch block |
Depolarization abnormalities | Minor: Low QRS voltages (<0.5 mV peak to peak) in limb leads, in the absence of obesity, emphysema, or pericardial effusion |
Ventricular arrhythmias | Minor: Frequent ventricular extrasystoles (>500 per 24 h), non-sustained, or sustained ventricular tachycardia with a right bundle branch block morphology (excluding the fascicular pattern) |
Family history/genetics | Major: -ACM confirmed in a first-degree relative who meets diagnostic criteria -ACM confirmed at autopsy or surgery in a first-degree relative -Identification of a pathogenic or likely pathogenic 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 years of age) due to suspected ACM in a first-degree relative -ACM confirmed pathologically or by diagnostic criteria in a second-degree relative |
Abbreviations: ACM, arrhythmogenic cardiomyopathy; CMR, cardiac magnetic resonance; LGE, late gadolinium enhancement; LV, left ventricle.