Prolonged QT interval in Rett syndrome
Prolonged QT interval in Rett syndrome
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DOI：
10.1136/adc.80.5.470
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发表时间：
1999-05-01
影响因子：
5.2
通讯作者：
Christodoulou, J
中科院分区：
文献类型：
--
作者：
Ellaway, CJ;Sholler, G;Christodoulou, J
雷特综合征是一种病因不明的严重神经发育障碍。先前曾描述过 Rett 综合征患者 QT 间期延长。为了研究 Rett 综合征中 QT 延长和心律失常的存在，我们前瞻性地对 34 名患有 Rett 综合征的女孩进行了心电图和 24 小时动态心电图监测。九名患者的校正 QT 值延长。与相似年龄范围的健康对照组相比，Rett 综合征患者的校正 QT 值明显更长。在 Rett 综合征队列中，临床严重程度并不是 QT 间期延长的预测因素。 QT 间期延长综合征是一种严重且可能致命的心脏疾病，所有患有 Rett 综合征的女孩都应予以考虑。
Rett syndrome is a severe neurodevelopmental disorder of unknown aetiology. A prolonged QT interval has been described previously in patients with Rett syndrome. To investigate QT prolongation and the presence of cardiac tachyarrhythmias in Rett syndrome electrocardiography and 24 hour Holter monitoring were performed prospectively in a cohort of 34 girls with Rett syndrome. The corrected QT value was prolonged in nine patients. Compared with a group of healthy controls of a similar age range, the patients with Rett syndrome had significantly longer corrected QT values. Clinical severity was not a predictor for prolonged QT intervals in the Rett syndrome cohort. The prolonged QT syndrome is a serious and potentially lethal cardiac disorder and should be considered in all girls with Rett syndrome.