Myocardial fibrosis imaging based on T1-mapping and extracellular volume fraction (ECV) measurement in muscular dystrophy patients: diagnostic value compared with conventional late gadolinium enhancement (LGE) imaging
Myocardial fibrosis imaging based on T1-mapping and extracellular volume fraction (ECV) measurement in muscular dystrophy patients: diagnostic value compared with conventional late gadolinium enhancement (LGE) imaging
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DOI：
10.1093/ehjci/jeu050
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发表时间：
2014-09-01
影响因子：
6.2
通讯作者：
Yilmaz, Ali
中科院分区：
文献类型：
--
作者：
Florian, Anca;Ludwig, Anna;Yilmaz, Ali
目的心肌纤维化导致扩张型心肌病是肌营养不良患者的主要死亡原因。基于造影前后T1标测的细胞外体积分数（ECV）测量有望检测早期“弥漫性”心肌纤维化，而这种纤维化无法通过基于晚期钆增强（LGE）的传统造影成像来描述。通过这项研究，我们评估了“正常”区域弥漫性心肌纤维化的存在，（LGE阴性）和“患病”结果27例Becker型肌营养不良症（BMD）患者中，LGE阳性心肌的检出率为100%（10/27）。（35 +/-12岁）和17名匹配的男性健康对照（33 +/-8岁）进行了心血管磁共振（CMR）研究，包括ECV测量和LGE成像。通过外部事件循环记录仪进行了脑卒中事件的动态监测。20例BMD患者（74%）表现出典型的下外侧存在LGE检测到心脏受累。12例患者（44%）左室射血分数受损，均为LGE阳性。BMD组的整体心肌ECV（29 +/- 6%）显著高于对照组（24 +/-2%，P = 0.001）。心脏受累患者的整体ECV较高与无心脏受累的BMD患者和对照组相比，LGE阳性节段（34 +/- 6%）和LGE阴性节段（28 +/- 6%）的局部ECV分别显著增加（24 +/- 3和24 +/-2%，P = 0.005）。心脏受累患者的整体ECV与左室射血分数（r =-0.629，P = 0.003）和LGE阳性节段数（r = 0.783，P = 0.001）显著相关。单因素分析显示，整体ECV（而非LGE本身的存在）与心肌纤维化事件显著相关（OR：1.97，CI：32.22-1.21，P = 0.032）。结论CMR测量ECV可用于评估心肌纤维化的总体程度，也可用于显示LGE图像上正常心肌区域的细微弥漫性纤维化。因此，心肌ECV是一个潜在的额外的定量工具，用于准确检测肌营养不良患者的心脏受累和危险分层。
Aim Cardiac involvement with progressive myocardial fibrosis leading to dilated cardiomyopathy is a major cause of death in muscular dystrophy patients. Extracellular volume fraction(ECV) measurement based on T1-mapping pre- and post-contrast promises the detection of early 'diffuse' myocardial fibrosis that cannot be depicted by conventional contrast- imaging based on late gadolinium enhancement (LGE). With this study, we evaluated the presence of diffuse myocardial fibrosis in regions of 'normal' (LGE-negative) and 'diseased' (LGE-positive) appearing myocardium as well as its relation to the extent of left ventricular(LV) dysfunction and the occurrence of arrhythmias in Becker muscular dystrophy(BMD) patients.Methods and results Twenty-seven BMD patients(35 +/- 12 years) and 17 matched male healthy controls(33 +/- 8 years) underwent cardiovascular magnetic resonance(CMR) studies including ECV measurement and LGE-imaging. Ambulatory monitoring of arrhythmic events was performed by means of an external event loop recorder. Twenty BMD patients(74%) demonstrated cardiac involvement as detected by typical inferolateral presence of LGE. Twelve patients(44%) had an impaired LV ejection fraction-all being LGE-positive. Global myocardial ECV was significantly higher in the BMD group(29 +/- 6%) compared with the control group(24 +/- 2%, P = 0.001). Patients with cardiac involvement demonstrated higher global ECV(31 +/- 6%) as well as significantly increased regional ECV not only in LGE-positive segments(34 +/- 6%), but also in LGE-negative segments(28 +/- 6%) compared with BMD patients without cardiac involvement and to controls, respectively (24 +/- 3 and 24 +/- 2%, P = 0.005). Global ECV in patients with cardiac involvement substantially correlated to LV ejection fraction(r = -0.629, P = 0.003) and to the number of LGE-positive segments(r = 0.783, P, 0.001). On univariable analysis, global ECV-but not the categorical presence of LGE per se--was significantly associated with arrhythmic events(OR: 1.97, CI: 32.22-1.21, P = 0.032).Conclusion ECV measurement byCMRis a useful tool in assessing the total extent of myocardial fibrosis as well as in depicting subtle diffuse fibrosis in areas of normal appearing myocardium on LGE-images. Thus, myocardial ECV is a potential additional quantitative tool for accurate detection of cardiac involvement and risk stratification in muscular dystrophy patients.