在威尔逊病时,细胞外体积增加,压力输液减少,缩功能恶化
Rebecka Steffen Johansson1, Csenge Fogarasi1, Peter Kellman2
1Department of Clinical Physiology, Karolinska University Hospital, and Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
概括
威尔逊病 (WD) 患者表现出早期心脏变化,如纤维化和功能减弱,即使没有症状. 心血管磁共振成像 (CMR) 揭示了这些微妙的迹象,强调了在WD中需要心脏监测的需要.
科学领域:
- 心脏病学 心脏病学
- 医疗成像医学成像
- 遗传学 是一个遗传学.
背景情况:
- 威尔逊病 (WD) 是由ATP7B基因突变引起的,导致铜积累和潜在的心脏问题.
- 在WD中观察到心脏参与,但无症状患者的潜在机制尚未完全理解.
研究的目的:
- 用先进的心血管磁共振成像 (CMR) 综合评估无症状WD患者的心肌.
- 评估心脏功能障碍,纤维化和心脏病患者的微血管变化的早期迹象.
主要方法:
- 在17名WD患者和17名健康对照中进行了多参数1.5TCMR.
- 技术包括cine,本地T1/T2,细胞外体积 (ECV),压力 perfusion 映射和晚期加多增强 (LGE).
- 分析的重点是心室功能,心肌应变, perfusion reserve,ECV和LGE的发现.
主要成果:
- 与对照组相比,WD患者表现出较低的LV喷射率和较差的LV全球周围应变.
- 减少压力输液和心肌输液储量 (MPR),以及增加心电量,在WD患者中观察到.
- 在RV插入点的晚期加多增强 (LGE) 在WD患者中普遍存在 (71%).
结论:
- 无症状的WD患者表现出早期心肌异常,包括扩散性纤维化,冠状动脉微血管功能障碍和缩功能受损.
- 这些发现表明,多参数CMR在检测WD中亚临床心脏参与方面具有实用性.
- 局限性包括小样本大小和缺少基因病理相关性.
关键词:
通过CMR多参数映射进行CMR多参数映射.在CMR perfusion 映射上进行CMR perfusion 映射.冠状动脉微血管功能障碍 冠状动脉微血管功能障碍压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion 压力 perfusion威尔逊病是威尔逊病的一种疾病.更多相关视频
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