先天性QT延长综合征尖端扭转型室性心动过速的发作方式

The mode and clinical implications of onset of spontaneous tosade de pointes in the congenital long QT syndrome

目的 研究先天性QT延长的尖端扭转型室性心动过速 (室速 )发作方式及其临床意义。方法 回顾性分析 5 5例因反复晕厥而确诊为先天性QT延长综合征病人的心电图 ,其中 16例记录到尖端扭转型室速开始发作的图形。结果 共记录到尖端扭转型室速 14 9阵 ,130阵为间隙依赖性。结论 间隙依赖性尖端扭转型室速曾经被认为是后天获得性QT延长综合征标志 。

Objective To study the mode and clinical implications of onset of spontaneous tosade de pointes in the congenital long QT syndrome. Methods We reviewed electrocardiograms (ECGs) of 55 patients with congenital QT syndrome for syncope. Documentation of the onset of tosade de pointes was available for 16 patients. All these patients had 'definitive long QT syndrome' by accepted clinical and ECG criteria. Results One hundren and forty-nine runs of tosade de pointes were documented in 16 patients,of whom,there were 130 runs of pause-dependent tosade de pointes. Conclusion Our results show that the pause-dependent tosade de pointes,which has been recognized as a hallmark of tosade de pointes in the acquired long QT syndrome,plays a major role in the genesis of tosade de pointes in the congenital long QT syndrome.