患有多变性心肌病的儿童的心肌功能工作:纵向评估和预后影响
Xander Jacquemyn1, Rebbeca Dryer2, Kyla Cordrey2
1The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA; Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
JACC. Advances
|June 18, 2025
概括
在儿科超性心肌病 (HCM) 中,心肌工作 (MW) 随着时间的推移而下降,并与不良结果相关. 然而,MW目前只是一个研究工具,并没有在全球纵向菌株上提供更好的风险分层.
科学领域:
- 心脏病学 心脏病学
- 儿童心脏病学 儿童心脏病学
- 生物医学工程 生物医学工程
背景情况:
- 心肌工作 (MW) 是已知预测成年超性心肌病 (HCM) 的不良结果的预测因素.
- 关于MW在儿科HCM中的作用和纵向变化的数据有限.
- 了解儿科MW对于风险分层和管理至关重要.
研究的目的:
- 描述患有HCM的儿科患者心肌功能 (MW) 的纵向变化.
- 评估MW指数与该人群中心血管不良结果之间的关联.
- 为了比较儿科HCM患者,家族史 (FH) 组和健康对照组之间的MW指数.
主要方法:
- 评估了74名儿科HCM患者 (平均年龄为11.9岁).
- 心肌工作指数 (GWI,GCW,GWE) 被测量并与家族史组和健康对照组进行比较.
- 死亡率,SCD,堕胎的SCD,适当的ICD释放和VT定义的不良结果的复合终点.
主要成果:
- 与对照组相比,儿科HCM患者的基线MW指数 (GWI,GCW,GWE) 显着较低.
- 在随访期间,HCM患者的全球工作指数 (GWI) 和全球建设性工作 (GCW) 显著下降.
- 较低的GWI和GCW与心血管不良事件的风险增加密切相关.
结论:
- 心肌功能在儿科HCM中纵向下降,并与不良结果有关.
- 目前的MW指数主要是研究工具,并且在风险分层方面没有超过全球纵向应变.
- 需要进一步的研究来完善MW在儿科HCM的临床效用.
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