对患有长QT综合征的试验者的受影响父母和兄弟姐妹的临床影响
J Kimbrough1, A J Moss, W Zareba
1Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Circulation
|August 2, 2001
概括
试验和LQTS的严重程度不能预测亲属的心脏事件. 对一级亲属的临床评估对于评估长QT综合征风险和指导管理至关重要.
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:
- 遗传性心脏疾病,如长QT综合征 (LQTS),需要进行家庭查.
- LQTS试验者的一级亲属经常携带这种情况.
- 评估家庭成员是全面LQTS管理的关键.
研究的目的:
- 评估LQTS试验试验的严重程度是否预测一级亲属心脏事件风险.
- 分析LQTS试验者的受影响父母和兄弟姐妹的临床过程.
主要方法:
- 分析了211名LQTS受试者和791名一级亲属的临床和心电图数据.
- 确定了受影响亲属心脏事件的危险因素.
- 实验对象及其受影响的亲属之间的临床严重程度进行了比较.
主要成果:
- 在父母或兄弟姐妹中,Proband LQTS的严重程度与心脏事件风险没有显著的相关性.
- 女性父母面临的第一个心脏事件的累积风险高于男性父母.
- 父母的心脏事件的风险因素是QTc持续时间和女性性别;兄弟姐妹的QTc.
结论:
- 普朗德的LQTS临床严重程度不是预测受影响一级亲属严重程度的可靠指标.
- 对亲属的个人风险评估对于有效的LQTS护理至关重要.
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