International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic.
1st Department of Internal Medicine/Cardioangiology, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
Orphanet J Rare Dis. 2019 Jan 9;14(1):10. doi: 10.1186/s13023-018-0986-0.
The progressive cardiomyopathy that develops in boys with Duchenne and Becker muscular dystrophy (DMD/BMD) is presumed to be a secondary consequence of the fibrosis within the myocardium. There are only limited data on using parametric imaging in these patients. The purpose of this study was to assess native T1 and extracellular volume (ECV) values in DMD patients.
The Czech population of males with DMD/BMD was screened. All eligible patients fulfilling the inclusion criteria were included. Forty nine males underwent cardiac magnetic resonance (MR) examination including T1 native and post-contrast mapping measurements. One DMD patient and all BMD patients were excluded from statistical analysis. Three groups were compared - Group D1 - DMD patients without late gadolinium enhancement (LGE) (n = 23), Group D2 - DMD patients with LGE (n = 20), and Group C - gender matched controls (n = 13).
Compared to controls, both DMD groups had prolonged T1 native relaxation time. These results are concordant in all 6 segments as well as in global values (1041 ± 31 ms and 1043 ± 37 ms vs. 983 ± 15 ms, both p < 0.05). Group D2 had significantly increased global ECV (0.28 ± 0.044 vs. 0.243 ± 0.013, p < 0.05) and segmental ECV in inferolateral and anterolateral segments in comparison with controls. The results were also significant after adjustment for subjects' age.
DMD males had increased native T1 relaxation time independent of the presence or absence of myocardial fibrosis. Cardiac MR may provide clinically useful information even without contrast media administration.
在杜兴氏肌营养不良症（DMD）和贝克肌营养不良症（BMD）的男孩中，逐渐发展的心肌病被认为是心肌纤维化的继发后果。在这些患者中，只有有限的数据用于参数成像。本研究的目的是评估 DMD 患者的心肌固有 T1 值和细胞外容积（ECV）值。
对捷克的 DMD/BMD 男性人群进行了筛查。所有符合纳入标准的合格患者均被纳入研究。49 名男性接受了心脏磁共振（MR）检查，包括心肌固有 T1 值和对比后测绘测量。一名 DMD 患者和所有 BMD 患者被排除在统计分析之外。三组进行比较：组 D1 - 无晚期钆增强（LGE）的 DMD 患者（n=23）；组 D2 - 有 LGE 的 DMD 患者（n=20）；组 C - 性别匹配的对照组（n=13）。
与对照组相比，两组 DMD 患者的心肌固有 T1 弛豫时间均延长。这些结果在所有 6 个节段以及整体值上都是一致的（1041±31ms 和 1043±37ms 与 983±15ms 相比，均 p<0.05）。与对照组相比，组 D2 的整体 ECV（0.28±0.044 与 0.243±0.013，p<0.05）和下外侧和前外侧节段的节段 ECV 明显增加。在调整了受试者的年龄后，结果仍然显著。
即使没有使用对比剂，DMD 男性的心肌固有 T1 弛豫时间也会增加，而与心肌纤维化的存在与否无关。心脏磁共振即使在不使用造影剂的情况下也可能提供有临床价值的信息。
