, Volume 6, Issue 3, pp 311-315

T-Wave Patterns Associated with the Hereditary Long QT Syndrome

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Mutations involving 6 different ion-channel genes have been identified in subjects with the hereditaryLong QT Syndrome. These gene mutations result in structural and functional changes in ion-channel proteins withresultant alterations in potassium and sodium repolarization currents that affect the morphologic features ofelectrocardiographic repolarization. This review highlights the genotype-phenotype associations related toventricular repolarization that have been reported in the LQTS literature, with particular focus on ECG T-wavepatterns in LQT1, LQT2, and LQT3 genotypes.