婴儿新发性心力衰竭:当病因诊断成为一个挑战时
Roger Esmel-Vilomara1,2,3, Lucía Riaza4,5, Paola Dolader6,4
1Paediatric Cardiology, Vall d'Hebron Hospital Campus, Barcelona, Spain. roger.esmel@gmail.com.
European journal of pediatrics
|October 16, 2023
概括
心脏磁共振成像 (CMR) 和内心肌组织活检 (EMB) 在心力衰竭的婴儿中显示炎症,但区分原因仍然具有挑战性. 包括EMB,CMR和基因检测在内的综合方法对于准确的诊断至关重要.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 心血管成像 - 心血管成像
- 炎症性心肌病 炎症性心肌病
背景情况:
- 内心肌活检 (EMB) 是心肌炎的黄金标准,但缺乏普遍接受.
- 2018年路易斯湖标准 (LLC) 在儿科患者的诊断效用需要进一步定义.
研究的目的:
- 为了评估心脏磁共振成像 (CMR) 与新发性心力衰竭的婴儿的定量映射.
- 评估EMB和CMR在检测炎症性心肌病并确定其病因方面的能力.
主要方法:
- 对接受CMR的婴儿进行前性分析,包括组织映射,EMB和遗传检测.
- 根据具有负遗传的炎症性心肌病 (疑似心肌炎) 与积极的遗传 (疑似扩张性心肌病) 进行分类.
- 分析了临床表现,心声学,素,NT-proBNP,EMB发现 (单核细胞,T淋巴细胞) 和CMR (包括晚期加多增强和组织映射).
主要成果:
- 所有患者都符合炎症性心肌病的EMB标准;在各组之间发现了炎症和的显著差异.
- 较高的热素水平与炎症增加相关.
- 无论是经典的CMR标准还是2018年易斯湖标准都没有区分这些群体;只有晚期的加多增强显示与心肌炎有关,而其他CMR参数和组织映射则没有.
- 在EMB检测到的炎症和CMR发现之间没有观察到相关性.
结论:
- 婴儿心脏功能障碍可能源于遗传因素或感染,通常涉及炎症.
- 虽然CMR对炎症敏感,但它可能无法区分心肌炎和婴儿扩张性心肌病.
- 一个全面的诊断策略是必不可少的,整合EMB (T淋巴细胞,瘤),CMR (LGE,组织映射),病毒检测,热素水平和遗传检测.
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