Long QT syndromes and torsade de pointes
Long QT syndromes and torsade de pointes
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DOI：
10.1016/s0140-6736(99)02107-8
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发表时间：
1999-11-06
期刊：
影响因子：
168.9
通讯作者：
Viskin, S
中科院分区：
文献类型：
--
作者：
Viskin, S
在长QT综合征（LQTS）中，离子通道的功能障碍损害心室复极并引发特征性室性心动过速：点扭转。LQTS的症状（晕厥或心脏骤停）是由这种心律失常引起的。在先天性LQTS中，编码离子通道的基因突变导致该通道故障。已经确定了6种基因型（LQT1至LQT6），并且正在尝试将不同的突变与临床症状和特异性治疗联系起来。在获得性LQTS中，通道功能障碍是由代谢异常或药物引起的。可能损害离子通道功能的药物名单不断扩大。此外，还认识到增加药物性扭转风险的因素（如女性、近期心率减慢或低钾血症）和心电图“警告信号”。最近的数据表明，获得性LQTS患者有一些潜在的心律失常倾向。突变导致先天性LQTS的“沉默”形式，在这种情况下，患者在暴露于进一步损害复极的药物之前不会出现心律失常，现在已经被发现。
In the long QT syndromes (LQTS), malfunction of ion channels impairs ventricular repolarisation and triggers a characteristic ventricular tachyarrhythmia: torsade de pointes. Symptoms in the LQTS (syncope or cardiac arrest) are caused by this arrhythmia. In congenital LQTS, mutations in the genes encoding for ion chanels cause this channel malfunction. Six genotypes (LQT1 to LQT6) have been identified, and attempts are being made to correlate different mutations with clinical signs and specific therapy. In acquired LQTS, channel malfunction is caused by metabolic abnormalities or drugs. The list of drugs that may impair ion-channel function expands continuously. Moreover, attributes that increase the risk for drug-induced torsade (eg, female sex, recent heart-rate slowing, or hypokalaemia) and electrocardiographic "warning signs" are recognised. Recent data suggest that patients with an acquired LQTS have some underlying predisposition to proarrhythmia. Mutations causing "silent" forms of congenital LQTS, in which the patient remains free of arrhythmias until exposed to drugs that further impair repolarisation, are now recognised.