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In the LQT2 form of long QT syndrome (LQTS), mutation sites are reported to correlate with clinical phenotypes in Caucasians, but the relationship in Asian patients remains unknown. The present study was designed to determine whether the location of KCNH2 mutations would influence the arrhythmic risk in LQT2 patients.

Mutation Site Dependent Variability of Cardiac Events in Japanese LQT2 Form of Congenital Long-QT Syndrome