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Non-contrast cardiovascular magnetic resonance detection of myocardial fibrosis in Duchenne muscular dystrophy
Journal of Cardiovascular Magnetic Resonance ( IF 6.4 ) Pub Date : 2021-04-29 , DOI: 10.1186/s12968-021-00736-1
Frank J Raucci 1, 2 , Meng Xu 3 , Kristen George-Durrett 1 , Kimberly Crum 1 , James C Slaughter 3 , David A Parra 1 , Larry W Markham 4 , Jonathan H Soslow 1
Affiliation  

Duchenne muscular dystrophy (DMD) leads to progressive cardiomyopathy. Detection of myocardial fibrosis with late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) is critical for clinical management. Due to concerns of brain deposition of gadolinium, non-contrast methods for detecting and monitoring myocardial fibrosis would be beneficial. We hypothesized that native T1 mapping and/or circumferential (εcc) and longitudinal (εls) strain can detect myocardial fibrosis. 156 CMRs with gadolinium were performed in 66 DMD boys and included: (1) left ventricular ejection fraction (LVEF), (2) LGE, (3) native T1 mapping and myocardial tagging (εcc-tag measured using harmonic phase analysis). LGE was graded as: (1) presence/absence by segment, slice, and globally; (2) global severity from 0 (no LGE) to 4 (severe); (3) percent LGE using full width half maximum (FWHM). εls and εcc measured using feature tracking. Regression models to predict LGE included native T1 and either εcc-tag or εls and εcc measured at each segment, slice, and globally. Mean age and LVEF at first CMR were 14 years and 54%, respectively. Global εls and εcc strongly predicted presence or absence of LGE (OR 2.6 [1.1, 6.0], p = 0.029, and OR 2.3 [1.0, 5.1], p = 0.049, respectively) while global native T1 did not. Global εcc, εls, and native T1 predicted global severity score (OR 2.6 [1.4, 4.8], p = 0.002, OR 2.6 [1.4, 6.0], p = 0.002, and OR 1.8 [1.1, 3.1], p = 0.025, respectively). εls correlated with change in LGE by severity score (n = 33, 3.8 [1.0, 14.2], p = 0.048) and εcc-tag correlated with change in percent LGE by FWHM (n = 34, OR 0.2 [0.1, 0.9], p = 0.01). Pre-contrast sequences predict presence and severity of LGE, with εls and εcc being more predictive in most models, but there was not an observable advantage over using LVEF as a predictor. Change in LGE was predicted by εls (global severity score) and εcc-tag (FWHM). While statistically significant, our results suggest these sequences are currently not a replacement for LGE and may only have utility in a very limited subset of DMD patients.

中文翻译:

杜氏肌营养不良症心肌纤维化的非对比心血管磁共振检测

杜氏肌营养不良症 (DMD) 导致进行性心肌病。通过心血管磁共振 (CMR) 检测具有晚期钆增强 (LGE) 的心肌纤维化对于临床管理至关重要。由于担心钆脑沉积,检测和监测心肌纤维化的非对比方法将是有益的。我们假设本机 T1 映射和/或圆周 (εcc) 和纵向 (εls) 应变可以检测心肌纤维化。在 66 名 DMD 男孩中进行了 156 次钆 CMR,包括:(1) 左心室射血分数 (LVEF),(2) LGE,(3) 天然 T1 标测和心肌标记(使用谐波相位分析测量的 εcc-tag)。LGE 被分级为:(1)按段、切片和全局的存在/不存在;(2) 全局严重性从 0(无 LGE)到 4(严重);(3) 使用半高全宽 (FWHM) 的 LGE 百分比。εls 和 εcc 使用特征跟踪测量。预测 LGE 的回归模型包括原生 T1 和在每个段、切片和全局测量的 εcc-tag 或 εls 和 εcc。第一次 CMR 时的平均年龄和 LVEF 分别为 14 岁和 54%。全局 εls 和 εcc 强烈预测 LGE 的存在或不存在(分别为 OR 2.6 [1.1, 6.0],p = 0.029 和 OR 2.3 [1.0, 5.1],p = 0.049),而全局原生 T1 则没有。全局 εcc、εls 和本地 T1 预测全局严重性评分(OR 2.6 [1.4, 4.8],p = 0.002,OR 2.6 [1.4, 6.0],p = 0.002,和 OR 1.8 [1.1, 3.1],p = 0.025,分别)。εls 与严重性评分的 LGE 变化相关(n = 33, 3.8 [1.0, 14.2],p = 0.048),εcc-tag 与 FWHM 的 LGE 百分比变化相关(n = 34,OR 0.2 [0.1, 0.9], p = 0.01)。对比前序列预测 LGE 的存在和严重程度,在大多数模型中,εls 和 εcc 更具预测性,但与使用 LVEF 作为预测因子相比,没有明显的优势。LGE 的变化由 εls(全球严重程度评分)和 εcc-tag (FWHM) 预测。虽然具有统计学意义,但我们的结果表明,这些序列目前不能替代 LGE,并且可能仅适用于非常有限的 DMD 患者子集。
更新日期:2021-04-29
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