长时间QT综合征. 对328个家庭进行了前性纵向研究
A J Moss1, P J Schwartz, R S Crampton
1Department of Medicine, University of Rochester School of Medicine and Dentistry, N.Y.
Circulation
|September 11, 1991
概括
长QT综合征 (LQTS) 患者,特别是试验者,面临昏和死亡的高风险. 关键的风险因素包括QTc间隔,先前的心脏事件和心率,指导临床管理.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 临床医学 临床医学
背景情况:
- 长QT综合征 (LQTS) 是一种罕见的家族性疾病,其特点是心电图上的QT间隔延长.
- 它使个体易患心室动脉节律失常,昏迷和心脏突然死亡.
研究的目的:
- 研究患有LQTS的个体的临床特征和长期结果.
- 为了确定LQTS患者昏迷和死亡的危险因素.
主要方法:
- 对来自328个家庭的3,343个人进行前性研究,发现了LQTS病例.
- 分析临床数据,心电图参数 (QTc),心率和心脏事件史.
主要成果:
- 试验对象年轻,女性更频繁,昏,心脏骤停和特定心电图发现率更高 (QTc ≥0.50秒1/2).
- 节律失调性昏迷通常是由兴奋引发的;发作有时被误诊为发作.
- 在12岁时,50%的试验者经历了昏迷或死亡. 试验对象的注册后事件率明显高于其他家庭成员.
结论:
- 在LQTS中,QTc间隔,心脏事件史和心率是昏迷或50岁前死亡的独立预测因素.
- 这项研究阐明了关键的临床特征,危险因素和LQTS的自然过程.
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