Ranolazine for Congenital Long QT Syndrome Type III (LQT3): Experimental and Long-Term Clinical Data

摘要

BackgroundThe basic defect in long QT syndrome type-III (LQT3) is an excessive inflow of sodium current during phase-3 of the action potential caused by mutations in the SCN5A gene. Most sodium channel blockers reduce the early (peak) and late components of the sodium current (INa and INaL) but ranolazine preferentially reduces INaL. We therefore evaluated the effects of ranolazine in LQT3 caused by the D1790G mutation in SCN5A.