To fully realize the potential of hiPSCs for elucidating the mechanistic basis and complex pathophysiology of channelopathies, we generated hiPSC-CMs from a patient with LQT2 harboring a heterozygous mutation (c.157G>A) in the KCNH2 gene, resulting in a Gly53-to-Ser substitution within the PAS domain. The gene discussed is KCNH2; the disease is channelopathy.