However, while gene-elusive and PKP2 variants represent the majority of ARVC cases fulfilling 2010 TFC at the time of their first sustained VA, fewer than half of patients carrying variants in genes such as DSP, PLN, and FLNC do so.7,80–82 Patients with these genotypes represent a distinct ACM subpopulation, with biventricular and left-dominant phenotypes significantly differing from the classical RV dominant disease for which ARVC guidelines were developed. Here, DSP is linked to arrhythmogenic right ventricular cardiomyopathy.