一名携带KCNE1（G38S）基因且出现尖端扭转型室速的患者的复极动力学异常。
Abnormal repolarization dynamics in a patient with KCNE1(G38S) who presented with torsades de pointes.
作者信息
Yamaguchi Yoshiaki, Mizumaki Koichi, Hata Yukiko, Inoue Hiroshi
机构信息
Second Department of Internal Medicine, Graduate School of Medicine, University of Toyama, Toyama, Japan.
Clinical Research and Ethics Center, University of Toyama, Toyama, Japan.
出版信息
J Electrocardiol. 2016 Jan-Feb;49(1):94-8. doi: 10.1016/j.jelectrocard.2015.10.002. Epub 2015 Oct 14.
Risk of G38S, major KCNE1 polymorphism [KCNE1(G38S)], for long QT syndrome (LQTS) remains unclear. A 72-year-old woman was admitted with recurrent torsades de pointes (TdP). She had remarkable QT prolongation (corrected QT interval 568 ms) under conditions of hypokalemia and hypomagnesemia. After correction of this electrolytic imbalance, TdP was suppressed and metoprolol was started. The QT-RR slope in 24-hour Holter electrocardiogram was steep and this enhanced bradycardia-dependent QT prolongation was similar to that in LQTS. She carried KCNE1(G38S). Patients with KCNE1(G38S) could have similar potential risk of ventricular arrhythmia as with LQTS. Analysis of QT-RR relationship could also evaluate the latent arrhythmogenicity of KCNE1(G38S).
KCNE1基因主要多态性位点G38S [KCNE1(G38S)]导致长QT综合征（LQTS）的风险尚不清楚。一名72岁女性因反复发生尖端扭转型室速（TdP）入院。在低钾血症和低镁血症情况下，她出现了显著的QT间期延长（校正QT间期为568毫秒）。纠正这种电解质失衡后，TdP得到抑制，并开始使用美托洛尔。24小时动态心电图显示QT-RR斜率陡峭，这种增强的心动过缓依赖性QT间期延长与LQTS相似。她携带KCNE1(G38S)基因。携带KCNE1(G38S)基因的患者可能具有与LQTS患者相似的室性心律失常潜在风险。分析QT-RR关系也可以评估KCNE1(G38S)的潜在致心律失常性。
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