Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate : implications for gene-specific therapy

作者： Peter J. Schwartz , Silvia G. Priori , Emanuela H. Locati , Carlo Napolitano , Francesco Cantù

关键词: Heart disease 、 Heart rate 、 QT interval 、 Heart block 、 Internal medicine 、 Sodium channel blocker 、 Cardiology 、 Medicine 、 hERG 、 Long QT syndrome 、 Mexiletine

摘要: Background The genes for the long QT syndrome (LQTS) linked to chromosomes 3 (LQT3) and 7 (LQT2) were identified as SCN5A, cardiac Na+ channel gene, HERG, a K+ gene. These findings opened possibility of attempting gene-specific control ventricular repolarization. We tested hypothesis that interval would shorten more in LQT3 than LQT2 patients response mexiletine also increases heart rate. Methods Results Fifteen LQTS studied. Six treated with mexiletine, its effects on QTc measured. Mexiletine significantly shortened among (QTc from 535±32 445±31 ms, P<.005) but not 530±79 503±60 P=NS). (n=7) their rate much (n=4) 18 healthy subjects (9.45±3.3 versus 3.95±1.97 2.83±1.33, P<.05; data e...

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Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate : implications for gene-specific therapy

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Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate : implications for gene-specific therapy

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