在小儿高性心肌病中预测突然心脏死亡风险的验证模型
Anastasia Miron1, Myriam Lafreniere-Roula2, Chun-Po Steve Fan2
1Division of Cardiology (A.M., T.P., S.M.), Hospital for Sick Children, Toronto, Ontario, Canada.
Circulation
|May 19, 2020
概括
已开发并验证了患有高性心肌病的儿童突然心脏死亡 (SCD) 的新风险预测模型. 该模型确定了儿科患者的独特风险因素,改善了SCD预防策略.
科学领域:
- 心脏病学
- 遗传学
- 儿童医学
背景情况:
- 缩性心肌病 (HCM) 是儿童群体突然心脏死亡 (SCD) 的首要原因.
- 在患有HCM的儿童中制定有效的SCD预防策略至关重要.
研究的目的:
- 开发和验证诊断为高性心肌病的儿科患者的SCD风险预测模型.
- 为实施有针对性的SCD预防战略提供指导.
主要方法:
- 一项国际多中心观察队列研究包括18岁以下的异型阳性患者.
- 使用具有因果特异性危险回归的竞争风险模型来确定临床和遗传性SCD风险因素.
- 该模型在一个独立的外部队列 (SHaRe注册表) 中得到验证.
主要成果:
- 在572名儿科HCM患者中,SCD事件的累计发病率为9. 1%.
- 确定的关键风险预测因素包括诊断时的年龄,非持续性心室低心率,昏迷,特定的心脏测量 (隔膜和后壁直径z-score,左心房直径z-score),LV流出通道梯度和致病变体.
- 临床和临床/遗传模型显示出良好的预测准确性 (c- 统计值为 0. 75- 0. 76) 和验证性 (0. 71- 0. 72).
结论:
- 已经建立了一个有效的SCD风险预测模型,其准确度超过70%.
- 该模型包含儿科特异性风险因素,使其与成人模型区分开来.
- 这种工具可以增强临床实践指南和在患有HCM的儿童中植入可植入心脏变压器 (ICD) 的共享决策.
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