The model S4 indicated high ET-1 associated risk for all-cause mortality (HR: 2.14; 95% CI 1.56–2.94; p < 0.001), CV death (HR: 1.8; 95% CI 1.17–2.78; p = 0.008), non-CV death (HR: 2.46; 95% CI 1.56–3.9; p < 0.001) and SCD (HR: 2.48; 95% CI 1.25–4.94; p = 0.01). The gene discussed is EDN1; the disease is Schnyder corneal dystrophy.