在高氨酸性综合征中的心脏异常
Viviane Tiemi Hotta1,2, Rafael Ruas Nastari1, Gardênia da Silva Lobo Oishi1,2
1Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brasil.
Arquivos brasileiros de cardiologia
|November 28, 2024
概括
过敏氨基酸 (HE) 和过敏氨基酸综合征 (HES) 涉及高氨基酸计数和器官损伤. 心脏参与是常见的,诊断依赖于成像和活检,并根据原因量身定制治疗.
科学领域:
- 血液学 血液学 血液学
- 心脏病学 心脏病学
- 在瘤学瘤学.
背景情况:
- 超氨基酸性 (HE) 被定义为周围血液中氨基酸细胞的升高 (≥1500细胞/μL) 或骨髓中氨基酸性 (>20%).
- 过敏氨酸性综合征 (HES) 涉及带有器官损伤的HE,被分类为初级,二级或特异性.
- 心脏参与HES影响5-20%的患者,呈现为心肌炎或心肌病变,严重程度各不相同.
研究的目的:
- 概述高氨酸性综合征 (HES) 中心脏参与的诊断方法.
- 突出多模式成像在诊断HES相关心脏病的作用.
- 强调基于病因的治疗对HES的重要性.
主要方法:
- 与HES相关的心脏病的诊断依赖于多模式成像,主要是心声回声 (回声).
- 用对比剂进行的胸外心声学 (TTE) 有助于可视化;评估心肌变形指数,如纵向应变.
- 心脏磁共振成像 (CMR) 识别了心下内脏晚期加多增强;内心肌活检是黄金标准.
主要成果:
- 在HES中,心脏功能障碍与乙氨基酸水平没有直接相关.
- 心声学可以揭示减少的纵向应变,特别是在心室顶部 (反向的尖端节省).
- CMR发现包括心下内分泌物晚期加多增强,表明心肌损伤.
结论:
- 在HES中,心脏参与呈现于死髓,血栓和纤维化的阶段,表现为心力衰竭,心律失常或血栓栓塞.
- 多模式成像,特别是心声回声和CMR,对于诊断与HES相关的心脏病至关重要.
- 对HES心脏病的有效治疗取决于识别和解决潜在的病因.
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