Approximately 25% of inherited LQTS cases and almost all cases of acquired LQTS arising from pharmacological QTc prolongation are associated with the Kv11.1 cardiac voltage-gated K+ channel (also known as human Ether-à-go-go related gene; hERG), encoded by the KCNH2 gene (Tester and Ackerman, 2014). Here, KCNH2 is linked to familial long QT syndrome.