Azzu Alessia, Antonopoulos Alexios S, Krupickova Sylvia, Mohiaddin Zain, Almogheer Batool, Vlachopoulos Charalambos, Pantazis Antonis, Pennell Dudley J, Mohiaddin Raad H
CMR Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, SW3 6NP London, UK.
National Heart and Lung Institute, Imperial College London, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, Chelsea, SW3 6NP London, UK.
Eur Heart J Cardiovasc Imaging. 2023 Mar 21;24(4):503-511. doi: 10.1093/ehjci/jeac129.
Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD.
The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich's ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne's muscular dystrophy (BMD/DMD, n = 15), Duchenne's carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE.
3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.
心血管磁共振成像（CMR）对于检测神经肌肉疾病（NMDs）中的心脏受累情况具有重要价值。我们探讨了使用特征追踪（FT）-CMR进行二维和三维左心室（LV）心肌应变分析以检测NMD亚临床心脏受累的价值。
本研究对111例NMD患者进行了回顾性分析，包括线粒体细胞病（n = 14）、弗里德赖希共济失调（FA，n = 27）、强直性肌营养不良（n = 27）、贝克尔/杜兴肌营养不良（BMD/DMD，n = 15）、杜兴携带者（n = 6）或其他（n = 22），以及57名年龄和性别匹配的健康志愿者。通过FT-CMR评估双心室容积、心肌晚期钆增强（LGE）和左心室心肌变形，包括二维和三维整体圆周应变（GCS）、整体径向应变（GRS）、整体纵向应变（GLS）和扭转。与健康志愿者相比，NMD患者的二维GCS（P < 0.001）和二维GRS（短轴，P < 0.001）受损，但二维GRS长轴（P = 0.101）、二维GLS（P = 0.069）或扭转（P = 0.122）无显著差异。三维GRS、三维GCS和三维GLS值与对照组均有显著差异（所有P < 0.0001）。特别是，即使是没有明显心脏受累（即左心室扩张/肥厚、左心室射血分数降低或存在LGE）的NMD患者，其三维GRS、GCS和GLS与对照组相比也有显著受损（P < 0.0001）。三维GRS和GCS值与LGE的存在和模式显著相关，在透壁LGE患者中受损最严重。
三维FT CMR可在NMD患者出现替代性纤维化或心室重构之前检测到亚临床心肌病，这可能是早期检测心脏受累的一种有用的影像学生物标志物。
