通过心脏磁共振成像在患有杜氏肌肉发育不良的患者左心室相位功能
Taylor M Trussell1,2,3, Tyler Brown4,5,6, Elisa Marcuccio4,5,6
1Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, USA. Taylor.Trussell@cchmc.org.
Pediatric cardiology
|November 8, 2023
概括
在杜氏肌力发育不良 (DMD) 患者中,左心房 (LA) 功能受损,空气分数减少与年龄相关,心脏功能恶化. 应变标志物表明DMD心肌病中早期的LA遵守和放松问题.
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:
- 杜氏肌肉发育不良 (DMD) 是导致心脏病发病率和死亡率的主要原因.
- 尽管存在潜在的结构变化,但DMD中的左心房 (LA) 功能尚不清楚.
- 心脏磁共振成像 (CMR) 提供了一种非侵入性方法来评估LA功能.
研究的目的:
- 用心磁共振 (CMR) 成像来评估和比较杜申肌力发育不良症 (DMD) 患者的左心房 (LA) 阶段功能.
- 调查LA功能,患者年龄和DMD中左心室 (LV) 缩功能之间的关系.
- 用LA菌株参数识别DMD中心脏功能障碍的早期指标.
主要方法:
- 对107项DMD和79项正常CMR研究进行了回顾性审查.
- 分析LA应变测量 (水库,管道,增压) 和LA体积.
- 斯皮尔曼相关性分析用于评估变量之间的关系.
主要成果:
- 与对照组相比,DMD队列表现出较差的缩功能,较小的LA/LV体积和较大的LA排空分数.
- 在DMD患者中,LA排空分数随着年龄和缩功能下降而下降.
- 在DMD中,中度/严重的LV功能障碍与较低的LA排空分数和LA储,和导管应变受损有关.
结论:
- 功能保留的DMD患者的LA体积较小,LA排空分数高于对照组.
- 疾病进展和年龄与LA排空分数的降低和储和导管应变的早期损害有关.
- LA菌株标志物反映了LA遵守和早期LV放松,表明DMD心肌病变的恶化.
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