Electrocardiogram Abnormalities Suggest Aberrant Cardiac Conduction in Huntington's Disease.
Electrocardiogram Abnormalities Suggest Aberrant Cardiac Conduction in Huntington's Disease.
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心电图异常表明亨廷顿舞蹈病的心脏传导异常。
DOI：
10.1002/mdc3.12596
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发表时间：
2018
影响因子：
4
通讯作者：
Rosas,HDiana
中科院分区：
文献类型：
--
作者：
Stephen,ChristopherD;Hung,Judy;Schifitto,Giovanni;Hersch,StevenM;Rosas,HDiana
越来越多的证据表明，亨廷顿舞蹈病（HD）的影响已经超出了中枢神经系统。特别是，在转基因小鼠模型中描述了明显的心功能障碍，并建议在有症状的患者中，心脏受累可能是心源性猝死的独立风险。方法对参与多地点III期研究的590名早期症状（1期和2期）HD患者筛查时获得的标准12导联心电图（ECGs）进行分析。结果仅评估未服用药物或有潜在医疗条件的个体的心电图，心动过缓的患病率为28.3%（标记为5.8%），QRS延长4.9%，室内传导延迟3.4%，右束支传导阻滞1.3%，QTc延长3.7%。结论以传导异常为主要特征的心脏异常患者多于预期。脑室内传导异常可能导致心律失常的风险增加，并可能因服用延长QT间期的药物而加重。
BackgroundThere is increasing evidence that the effects of Huntington's disease (HD) extend beyond the central nervous system. In particular, significant cardiac dysfunction has been described in transgenic mouse models and suggested in symptomatic patients, in whom cardiac involvement could provide an independent risk for sudden cardiac death.MethodsStandard 12‐lead electrocardiograms (ECGs) obtained at screening from 590 early symptomatic (Stage 1 and 2) HD patients participating in a multi‐site Phase III study were analyzed.ResultsEvaluating only those ECGs in individuals not on medications or with potentially contributing medical conditions, the prevalence of bradycardia was 28.3% (marked in 5.8%), prolonged QRS 4.9%, intraventricular conduction delay 3.4%, right bundle branch block 1.3%, and QTc prolongation 3.7%.ConclusionSignificant cardiac abnormalities, characterized primarily by conduction abnormalities, were found in a larger than expected number of patients. Abnormal intraventricular conduction may lead to increased risk for arrhythmia and may be compounded by prescription of QT‐prolonging medications.