症状呈现会影响小儿限制性心肌病的结果
Melissa Lorenzo1, Aine Lynch1, Jenna Ashkanase2
1Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
Frontiers in pediatrics
|November 6, 2023
概括
患有限制性心肌病 (RCM) 的儿童在诊断时表现出症状,预后更差. 早期识别症状对于儿童RCM患者及时进行心脏移植至关重要.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 心肌病研究研究 心肌病研究
- 移植医学 移植医学 移植医学
背景情况:
- 儿童的限制性心肌病 (RCM) 通常带来不良预后,往往导致死亡或心脏移植在两年内.
- 由于不断发展的查实践,早期诊断RCM正变得越来越普遍,突出了优化移植时间的必要性.
研究的目的:
- 为了比较患有有临床症状和没有临床症状的RCM儿童之间的结果.
- 确定儿科RCM患者诊断时症状的预后意义.
主要方法:
- 在2001年至2018年期间诊断出25名儿科RCM患者的回顾性队列研究.
- 在症状和无症状组之间比较移植时间,死亡和心脏不良事件的复合.
主要成果:
- 无症状患者的2年无移植存活率 (57%对17%,p=0.03) 和无心脏事件存活率 (71%对25%,p=0.01) 显著改善.
- 呈现时的心脏症状是心脏移植或死亡的独立风险因素 (HR 5.17,p=0.02).
结论:
- 患有症状的儿科RCM患者面临着明显更差的无移植和无心脏事件的生存率.
- 心脏症状是一个关键的负面预后标志物,需要紧急考虑心脏移植清单.
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