低血压左心综合征的分阶段重建手术后的长期生存和患者报告的结果
J William Gaynor1, Marlene Mahle1, Richard F Ittenbach2
1Division of Cardiothoracic Surgery, Department of Surgery, Children's Hospital of Philadelphia, and the Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Journal of the American College of Cardiology
|June 18, 2025
概括
不到三分之一的低可塑性左心综合征 (HLHS) 患者在没有移植的情况下存活了35年. 虽然最近的生存率并没有改善,但一些幸存者报告了很好的长期生活质量.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 遗传性心脏病研究 遗传性心脏病研究
- 手术结果分析手术结果分析
背景情况:
- 低形成性左心综合征 (HLHS) 的存活率有所改善,但仍然低于其他先天性心脏缺陷.
- 对HLHS患者的长期结局数据有限.
研究的目的:
- 评估HLHS患者的长期存活率.
- 为了确定影响HLHS生存的患者因素.
- 评估成年人的功能状态和生活质量 (QOL).
主要方法:
- 对2,012名患有HLHS的新生儿进行分析,这些新生儿接受了诺伍德或混合程序 (1984-2023年).
- 从医疗记录中提取的数据;生命状况通过多个来源得到确认.
- 通过患者自我报告评估的成人功能结果和QOL.
主要成果:
- 在HLHS患者的35年无移植生存率为31.0%.
- 近年来,生存率并没有显著改善.
- 一部分幸存者报告说,他们的整体健康状况良好至极佳,以及QOL.
结论:
- 在没有心脏移植的情况下,只有不到三分之一的HLHS患者能活到35岁.
- 对于HLHS的长期存活率已经停滞不前,最近没有任何改善.
- 在一些幸存者中观察到一种"高性能"的Fontan表型,报告了良好的QOL.
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