CNVs involving single or multiple exons of the major channelopathy genes, KCNQ1, KCNH2, SCN5A, and RYR2, uncommonly serve as the pathogenic basis of dominantly inherited arrhythmia syndromes such as long QT syndrome, Brugada syndrome, and CPVT (3, 8, 9, 11, 17–19). Here, KCNQ1 is linked to channelopathy.