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

Table 1 Characteristics of arrhythmogenic ventricular cardiomyopathy

	Classic right dominant form (ARVC/D)	Left dominant form
12-lead surface ECG	Intraventricular conduction delay in V1-V3	Leftward QRS axis (< 0°)
	QRS complex prolongation V1-V3	ε like waves in inferior or lateral leads
	ε wave in V1-V3	LBBB
	(Incomplete) RBBB	Inverted T-waves in infero-lateral leads
	Inverted T-waves in V1-V3	Inverted T-waves V1-6 with biventricular involvement
	Inverted T-waves in V1-V6 with biventricular involvement	-
	ST elevation in V1-V3	-
	Poor R wave progression
Signal-averaged ECG	Late potentials	-
Arrhythmia	PVC/VT of LBBB configuration	PVC/VT of RBBB configuration
Ventricular volumes	Mild to severe RV-dilation ± dysfunction	Mild to severe LV-dilation ± dysfunction
RV/LV volume ratio	≥ 1.2, increases with disease expression	< 1.0
Other imaging abnormalities	Regional wall motion abnormalities in RV	Regional wall motion abnormalities in LV
	RV aneurysms	Non-compacted appearance
	Fat/LGE in RV myocardium	LGE in the subepicardial and midwall LV myocardium
Genetics	Affected genes currently known to be associated with AVC	Association with TMEM43 and phospholamban mutations[1]

Adapted from Jacoby et al[84]. ARVC/D: Arrhythmogenic right ventricular cardiomyopathy/dysplasia; ECG: Electrocardiogram; ε: Epsilon; LBBB: Left bundle branch block; LGE: Late gadolinium enhancement; LV: Left ventricle; PVC: Premature ventricular contraction; RBBB: Right bundle branch block; RV: Right ventricle; VT: Ventricular tachycardia; AVC: Arrhythmogenic ventricular cardiomyopathy; TMEM43: Transmembrane protein 43.