KCNH2 and familial long QT syndrome: In an Italian family Crotti et al. studied a small LQT2 family and reported a KCNH2 mutation (A1116V) and the T allele of SNP K897T on the non-mutant allele (i.e. in trans orientation) in one severe, symptomatic LQTS patient, whereas 3 relatives with A1116V alone were asymptomatic and showed mild QTc prolongation [20].