Migdalovich et al. analysed data of 1166 subjects with genetically confirmed KCNH2 variants and demonstrated that women experienced a high rate of life-threatening events regardless of mutation location (pore-loop: 35%, non-pore loop: 23%), whereas in men, the rate of aborted cardiac arrest or SCD was higher among those with pore-loop mutations (28%). This evidence concerns the gene KCNH2 and Schnyder corneal dystrophy.