Analyses from Casella et al. 32 and Aquaro et al. 41 reported a significant underprediction ARVC risk calculator-derived VA risk in patients with a left-dominant ARVC phenotype, while Protonotarios et al. 26 showed the ARVC risk calculator over-predicting arrhythmic risk in patients with a P/LP variants in the DSP gene fulfilling the conditions for ARVC risk calculator usage. Here, DSP is linked to Arrhythmogenic right ventricular dysplasia.