Arrhythmic risk stratification in arrhythmogenic cardiomyopathy: new predictors for left-sided variants?

This editorial refers to ‘Prediction of ventricular arrhythmia in phospholamban p.Arg14del mutation carriers: reaching the frontiers of individual risk prediction’, by T.E. Verstraelen et al. doi:10.1093/eurheartj/ehab294. Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disease characterized by fibrofatty myocardial replacement that underlies structural and functional ventricular abnormalities and life-threatening ventricular arrhythmias potentially responsible for sudden cardiac death (SCD).1,2 The original disease phenotype characterized by a predominant right ventricular involvement was termed ‘arrhythmogenic right ventricular cardiomyopathy’ (ARVC); however, the increasing identification of biventricular and left-dominant [also referred to as ‘arrhythmogenic left ventricular cardiomyopathy’ (ALVC)] forms has led to the use of the designation of ACM, which better defines the broad spectrum of the disease phenotypic variants also involving the left ventricle.2,3 In ∼50% of patients with ARVC, pathogenic mutations of genes encoding desmosomal proteins such as plakophilin (PKP2), desmoplakin (DSP), desmoglein (DSG2),...