应用心血管磁共振 native T1 mapping 对杜氏肌营养不良症心肌受累的全局、节段和分层分析。
Global, segmental and layer specific analysis of myocardial involvement in Duchenne muscular dystrophy by cardiovascular magnetic resonance native T1 mapping.
机构信息
Department of Radiology, Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, 20# Section 3 South Renmin Road, Chengdu, 610041, China.
Department of Radiology, West China Hospital, Sichuan University, Chengdu, China.
出版信息
J Cardiovasc Magn Reson. 2021 Oct 14;23(1):110. doi: 10.1186/s12968-021-00802-8.
BACKGROUND
Progressive cardiomyopathy accounts for almost all mortality among Duchenne muscular dystrophy (DMD) patients. Thus, our aim was to comprehensively characterize myocardial involvement by investigating the heterogeneity of native T1 mapping in DMD patients using global and regional (including segmental and layer-specific) analysis across a large cohort.
METHODS
We prospectively enrolled 99 DMD patients (8.8 ± 2.5 years) and 25 matched male healthy controls (9.5 ± 2.5 years). All subjects underwent cardiovascular magnetic resonance (CMR) with cine, T1 mapping and late gadolinium enhancement (LGE) sequences. Native T1 values based on the global and regional myocardium were measured, and LGE was defined.
RESULTS
LGE was present in 49 (49%) DMD patients. Global native T1 values were significantly longer in LGE-positive (LGE +) patients than in healthy controls, both in basal slices (1304 ± 55 vs. 1246 ± 27 ms, p < 0.001) and in mid-level slices (1305 ± 57 vs. 1245 ± 37 ms, p < 0.001). No significant difference in global native T1 was found between healthy controls and LGE-negative (LGE-) patients. In segmental analysis, LGE + patients had significantly increased native T1 in all analyzed segments compared to the healthy control group. Meanwhile, the comparison between LGE- patients and healthy controls showed significantly elevated values only in the basal anterolateral segment (1273 ± 62 vs. 1234 ± 40 ms, p = 0.034). Interestingly, the epicardial layer had a significantly higher native T1 in LGE- patients than in healthy controls (p < 0.05), whereas no such pattern was noticed in the global myocardium. Epicardial layer native T1 resulted in the highest diagnostic performance for distinguishing between healthy controls and DMD patients in receiver operating curve analyses (area under the curve [AUC] 0.84 for basal level and 0.85 for middle level) when compared to global native T1 and endocardial layer native T1.
CONCLUSIONS
Myocardial regional native T1, particularly epicardial native T1, seems to have potential as a novel robust marker of very early cardiac involvement in DMD patients.
TRIAL REGISTRATION
Chinese Clinical Trial Registry ( http://www.chictr.org.cn/index.aspx ) ChiCTR1800018340, 09/12/2018, Retrospectively registered.
背景
进行性心肌病是杜氏肌营养不良症（DMD）患者死亡的主要原因。因此，我们的目的是通过对大量患者队列进行全局和局部（包括节段和分层特异性）分析，全面描述心肌受累情况。
方法
我们前瞻性纳入 99 名 DMD 患者（8.8±2.5 岁）和 25 名匹配的男性健康对照者（9.5±2.5 岁）。所有患者均接受心血管磁共振（CMR）检查，包括电影、T1 映射和晚期钆增强（LGE）序列。测量基于全局和局部心肌的 T1 值，并定义 LGE。
结果
49 名（49%）DMD 患者存在 LGE。与健康对照组相比，LGE 阳性（LGE+）患者的 T1 值在基础层面（1304±55 比 1246±27ms，p<0.001）和中层层面（1305±57 比 1245±37ms，p<0.001）均显著延长。LGE-患者的 T1 值与健康对照组无显著差异。节段分析显示，与健康对照组相比，所有分析节段的 LGE+患者的 T1 值均显著升高。同时，与健康对照组相比，LGE-患者仅在前外侧基底节段的 T1 值显著升高（1273±62 比 1234±40ms，p=0.034）。有趣的是，与健康对照组相比，LGE-患者的心外膜层 T1 值明显升高（p<0.05），而在整体心肌中未见此模式。与整体 T1 值和心内膜层 T1 值相比，心外膜层 T1 值在接受者操作特征曲线分析中区分健康对照组和 DMD 患者的诊断性能最高（基底水平 AUC 为 0.84，中层水平 AUC 为 0.85）。
结论
心肌局部 T1 值，特别是心外膜 T1 值，似乎有可能成为 DMD 患者心脏早期受累的一种新的可靠标志物。
临床试验注册
中国临床试验注册中心（http://www.chictr.org.cn/index.aspx）ChiCTR1800018340，2018 年 9 月 12 日，回顾性注册。
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