Interestingly, Maltese (2016) reported another Russian patient with clinically evident LQTS, QTc up to 530 ms, syncope, and ICD implanted, who was also the double-heterozygous carrier of the p.Arg82Gln variant in the KCNJ2 gene and additional low-penetrant allele p.Arg176Trp in the KCNH2 gene [23]. Here, KCNH2 is linked to familial long QT syndrome.