Table 1

Most common differential diagnostic considerations for ARVC

Differential diagnosis	Comparison of clinical features
Cardiac sarcoidosis	Similarities with ARVC: focal myocardial lesions, (regional) ventricular dysfunction, arrhythmias and LGE with non-ischaemic pattern. Contrasting with ARVC: non-familial pattern, atrioventricular conduction delay, extracardiac manifestations and predominant intraventricular septal involvement.
Myocarditis	Similarities with ARVC: non-ischaemic LGE and arrhythmias. Contrasting with ARVC: history of viral prodromes, imaging findings suggesting myocardial oedema (acute phase) as well as pericardial involvement.
Dilated cardiomyopathy	Similarities with ARVC: familial pattern, phenotype may mimic ARVC/ACM with LV involvement. Contrasting with ARVC: ventricular arrhythmias predominantly in context of impaired ventricular structure/function, usually preceded by heart failure.
Uhl’s anomaly	Similarities with ARVC: loss of RV myocardium and RV dilatation. Contrasting with ARVC: non-familial, RV birth defect, deficiency of myocardium appearing as ‘parchment’, symptoms early childhood and primarily heart failure.
Brugada syndrome	Similarities with ARVC: ventricular arrhythmias and pseudo right bundle branch block. Contrasting with ARVC: ventricular arrhythmias predominantly at rest and structural abnormalities absent.
Athlete’s heart	Similarities with ARVC: cardiac remodelling may mimic ARVC and exercise accelerates structural modifications. Contrasting with ARVC: reversible, balanced biventricular dilatation and hypertrophy, no dysfunction and no regional wall motion abnormalities.
Idiopathic RVOT VT	Similarities with ARVC: VTs with LBBB inferior axis morphology. Contrasting with ARVC: benign prognosis, curative catheter ablation and structural/ECG abnormalities usually absent.

ACM, arrhythmogenic cardiomyopathy; ARVC, Arrhythmogenic right ventricular cardiomyopathy; LBBB, left bundle branch block; LGE, late gadolinium enhancement; LV, left ventricular; RV, right ventricular; RVOT, RV outflow tract; VT, ventricular tachycardia.