Arrhythmogenic ventricular cardiomyopathy: A paradigm shift from right to biventricular disease

Table 4 Revised (2010) task force criteria for diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia, adapted from Marcus et al[92]

	Structural alterations
Major	TTE regional RV akinesia, dyskinesia, or aneurysm and 1 of the following criteria (end diastole)
	PLAX RVOT ≥ 32 mm [(PLAX/BSA) ≥ 19 mm/m2]
	PSAX RVOT ≥ 36 mm [(PSAX/BSA) ≥ 21 mm/m2]
	Or RV fractional area change ≤ 33%
	CMR regional RV akinesia, dyskinesia, or dyssynchronous RV contraction and 1 of the following criteria (end diastole)
	RV end-diastolic volume/BSA ≥ 110 mL/m2 (♂) or ≥ 100 mL/m2 (♀)
	Or RV ejection fraction ≤ 40%
	RV angiography regional RV akinesia, dyskinesia, or aneurysm
Minor	TTE regional RV akinesia, or dyskinesia and 1 of the following criteria (end diastole)
	PLAX RVOT ≥ 29-31mm [(PLAX/BSA) ≥ 16-18 mm/m2]
	PSAX RVOT ≥ 32-35 mm [(PSAX/BSA) ≥ 18-20 mm/m2]
	RV fractional area change > 33%-39%
	CMR regional RV akinesia, dyskinesia, or dyssynchronous RV contraction and 1 of the following criteria (end diastolic)
	RV end-diastolic volume/BSA ≥ 100-109 mL/m2 (♂) or ≥ 90-99 mL/m2 (♀)
	Or RV ejection fraction > 40%-44%
	Histopathology (endomyocardial biopsy)
Major	Residual myocytes < 60% by morphometric analysis with fibrous replacement of the RV free wall myocardium ≥ 1 sample, with or without fatty replacement
Minor	Residual myocytes 60%-75% by morphometric analysis with fibrous
	Replacement of the RV free wall ≥ 1 sample
	Repolarization abnormalities (> 14 years of age)
Major	T-wave inversions V1-V3 or beyond (in absence of complete RBBB)
Minor	T-wave inversions V1-V2 or V4-V6 (in absence of complete RBBB)
	T-wave inversions V1-V4, if complete RBBB present
	Depolarization abnormalities
Major	Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T-wave) in V1 to V3
Minor	SAECG with late potentials (if QRS complex on standard surface ECG < 110 ms) or terminal activation duration of QRS ≥ 55 ms in V1, V2 or V3
	Arrhythmias
Major	VT of LBBB morphology with superior axis
Minor	VT of RVOT configuration, LBBB morphology with inferior axis or of unknown axis
	> 500 PVC per 24 h (holter)
	Family history
Major	ARVC/D in a first-degree relative who meets current TFC
	ARVC/D confirmed pathologically at autopsy or surgery in a first-degree relative
	Identification of a pathogenic mutation categorized associated with ARVC/D in an index patient
Minor	Suspected ARVC/D in a first-degree relative-premature SCD (< 35 years of age) due to suspected ARVC/D in a first-degree relative
	ARVC/D confirmed pathologically or by current TFC in second-degree relatives

Definite diagnosis: two major or one major and two minor criteria or four minor from different categories; Borderline diagnosis: one major and one minor or three minor criteria from different categories; Possible diagnosis: one major or two minor criteria from different categories. BSA: Body surface area; CMR: Cardiac magnetic resonance tomography; LV: Left ventricle; PLAX: Parasternal long-axis view; PSAX: Parasternal short-axis view; RBBB: Right bundle branch block; RVOT: Right ventricular outflow tract; RV: Right ventricle, TTE: Transthoracic echocardiogram, PVC: Premature ventricular contraction VT: Ventricular tachycardia; SAECG: Signal-averaged electrocardiographic; LBBB: Left bundle branch block; ARVC/D: Arrhythmogenic right ventricular cardiomyopathy/dysplasia; TFC: Task force criteria; SCD: Sudden cardiac death.