Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent?
Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent?
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DOI：
10.1136/heart.83.6.661
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发表时间：
2000-06-01
期刊：
影响因子：
5.7
通讯作者：
Barron, HV
中科院分区：
文献类型：
--
作者：
Viskin, S;Fish, R;Barron, HV
目的：探讨先天性长QT间期综合征和自发性室性快速性心律失常患者发生间歇依赖性尖端扭动的频率及其预测因素。设计：对有关先天性长QT间期的文献进行综述。结果：在文献中发现62例先天性长QT间期综合征患者的自发性尖端扭转性室性心律失常。大多数记录的心律失常(74%)是“停顿依赖的”；其中82%的停顿比基本周期长度长100ms。年龄、性别与心律失常的发生方式相关。3岁以下婴幼儿心律失常与间歇依赖无关，而女性与间歇依赖性扭体反应相关。经多因素分析，年龄是尖端扭转性心律失常发病方式的唯一独立预测因素。结论现有数据表明，先天性长QT间期综合征中大多数自发性心律失常是停顿依赖的。没有停顿的尖端扭曲症似乎仅限于有严重疾病形式的患者亚组，如有症状的婴儿。这些发现对于使用心脏起搏预防心律失常具有重要意义。
Objective-To determine the frequency and predictors of pause dependent torsade de pointes among patients with the congenital long QT syndrome and spontaneous ventricular tachyarrhythmias.Design-The literature on the "congenital long QT" was reviewed. Articles with illustrations demonstrating the onset of spontaneous polymorphic ventricular arrhythmias in the absence of arrhythmogenic drugs were included.Results-Illustrations of 62 spontaneous episodes of torsade de pointes among patients with congenital long QT syndrome were found in the literature. The majority (74%) of documented arrhythmias were "pause dependent"; 82% of these pauses were longer than the basic cycle length by > 100 ms. Age and sex correlated with the mode of arrhythmia initiation. Arrhythmias in infants (less than or equal to 3 years old) were not pause dependent, while female sex correlated with pause dependent torsade. Using multivariate analysis, age was the only independent predictor of the mode of onset of torsade de pointes.Conclusion-Available data suggest that the majority of spontaneous arrhythmias in the congenital long QT syndrome are pause dependent. Torsade de pointes that is not preceded by pauses appears to be limited to patient subgroups with severe forms of the disease, like symptomatic infants. These findings have important implications regarding the use of cardiac pacing for arrhythmia prevention.