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杜氏肌营养不良症中的心肌损伤:通过心脏磁共振体素内不相干运动进行评估。

Myocardial injury in Duchenne muscular dystrophy: assessment via cardiac magnetic resonance intra-voxel incoherent motion.

作者信息

Yamamoto Atsushi, Nagao Michinobu, Ishigaki Keiko, Shichiji Minobu, Kihara Yuki, Sakai Akiko, Minami Yuichiro, Goto Yasuhiro, Yoneyama Masami, Sakai Shuji, Yamaguchi Junichi

机构信息

Department of Cardiology, Tokyo Women's Medical University, Tokyo, 162-8666, Japan.

Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University, 8-1, Kawadacho, Shinjuku-ku, Tokyo, 162-8666, Japan.

出版信息

Heart Vessels. 2025 Jul 21. doi: 10.1007/s00380-025-02580-0.

Abstract

Duchenne muscular dystrophy (DMD) is a progressive myopathy caused by a mutation in the dystrophin gene. Cardiac disease is currently the leading cause of death in patients with DMD; thus, early diagnosis and management of cardiomyopathy are essential. Intra-voxel incoherent motion (IVIM) analysis provides quantitative values from diffusion-weighted imaging (DWI) and may serve as a novel index for evaluating myocardial properties in DMD. We conducted IVIM analysis in patients with DMD and compared their results with those of healthy volunteers to confirm that IVIM analysis can detect myocardial damage more effectively than conventional imaging methods. Patients with DMD who underwent cardiac magnetic resonance IVIM were enrolled. D and F values were measured using DWI of the left ventricle. Six healthy volunteers served as the control group. Twelve male patients with DMD were enrolled (median age, 14 years). Creatinine kinase levels were elevated, brain natriuretic peptide values remained within the normal range, and troponin T levels were only mildly elevated. The D value in patients with DMD was significantly higher than that in healthy volunteers, indicating increased diffusion in the myocardium (2.59 vs. 1.85, p = 0.0057); however, the F value was comparable between the two groups (0.62 vs. 0.61, p = 0.60). The D and F values from IVIM analysis provided a more detailed reflection of myocardial properties. Myocardial diffusion in patients with DMD was elevated compared with that in healthy volunteers.

摘要

杜氏肌营养不良症(DMD)是一种由肌营养不良蛋白基因突变引起的进行性肌病。心脏疾病是目前DMD患者的主要死因;因此,心肌病的早期诊断和管理至关重要。体素内不相干运动(IVIM)分析可从扩散加权成像(DWI)中提供定量值,并可作为评估DMD心肌特性的新指标。我们对DMD患者进行了IVIM分析,并将其结果与健康志愿者的结果进行比较,以证实IVIM分析比传统成像方法能更有效地检测心肌损伤。纳入接受心脏磁共振IVIM检查的DMD患者。使用左心室的DWI测量D值和F值。六名健康志愿者作为对照组。纳入12名男性DMD患者(中位年龄14岁)。肌酸激酶水平升高,脑钠肽值保持在正常范围内,肌钙蛋白T水平仅轻度升高。DMD患者的D值显著高于健康志愿者,表明心肌扩散增加(2.59对1.85,p = 0.0057);然而,两组之间的F值相当(0.62对0.61,p = 0.60)。IVIM分析的D值和F值更详细地反映了心肌特性。与健康志愿者相比,DMD患者的心肌扩散升高。

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