In contrast, among ARVC cases, mutations most frequently localized to PKP2 (31.6% [29.2–34.1]), DSG2 (7.4% [6.0–8.8]), DSP (3.2% [2.3–4.2]), and TMEM43 (2.4% [1.6–3.2]). Here, DSP is linked to arrhythmogenic right ventricular cardiomyopathy.