Department of Medicine, Department of Cardiology, University of Würzburg, Oberdürrbacherstrasse 6, Würzburg, Germany.
Eur Heart J. 2013 Jun;34(21):1587-96. doi: 10.1093/eurheartj/eht098. Epub 2013 Mar 21.
This cross-sectional study aimed to analyse myocardial deformation in patients with Fabry disease (FD) in order to evaluate speckle tracking as a method for non-invasive determination of myocardial fibrosis. Myocardial fibrosis is common in Fabry cardiomyopathy and is associated with disease progression and severe prognosis.
In 101 consecutive Fabry patients (39.8 ± 12.9 years; 42 males), the quantitative measurement of myocardial fibrosis with magnetic resonance imaging was compared with regional myocardial deformation assessed by speckle-tracking imaging. Patients were analysed in relation to per cent of late-enhancement (LE)-positive areas of left-ventricular (LV) mass. Fifty-two patients (51%) displayed LE with a mean volume of 1.2 ± 1.8% of total LV mass. Predominantly basal lateral and posterior segments were affected. Patients with LE had lower global systolic longitudinal strain than those without (LE -14.8 ± 3.5% and -18.9 ± 2.1%, respectively; P < 0.001). Loss of global deformation, quantified by speckle tracking, was predominantly caused by basal posterior (P = 0.049) and lateral (P = 0.005) segments and global systolic strain correlated with the amount of LE (r = 0.543; P < 0.001). Patients with severe LE (>2%, n = 22) showed the lowest deformation values (-5.9 ± 8.4%) in basal postero-lateral segments when compared with those with mild (<2%; n = 30, -7.1 ± 7.5%) or no LE (n = 49, -16.3 ± 3.3%). These changes were accompanied by thinning of the posterior wall and a decrease in diastolic function, whereas ejection fraction and LV end-diastolic diameter were not different. Receiver operating characteristic analysis revealed that the systolic strain of basal postero-lateral segments was the most powerful predictor to distinguish between patients with and without LE (sensitivity = 90%; specificity = 97%, area under the curve = 0.913; P < 0.001).
Late enhancement is associated with lower longitudinal strain in the fibrotic wall segments. Speckle tracking can be used as a tool for the indirect evaluation of LE in FD.
本横断面研究旨在分析法布瑞氏病（FD）患者的心肌变形，以评估斑点追踪技术作为一种非侵入性确定心肌纤维化的方法。心肌纤维化在法布瑞心肌病中很常见，与疾病进展和严重预后相关。
在 101 例连续的 FD 患者（39.8±12.9 岁；42 名男性）中，通过磁共振成像对心肌纤维化进行定量测量，并通过斑点追踪成像评估区域性心肌变形。患者根据左心室（LV）质量的晚期增强（LE）阳性区域的百分比进行分析。52 例（51%）患者的 LV 质量有 LE，平均体积为 1.2±1.8%。主要受累节段为基底外侧和后段。有 LE 的患者的整体纵向收缩应变低于无 LE 的患者（LE：-14.8±3.5%和-18.9±2.1%；P<0.001）。通过斑点追踪术量化的整体变形丧失主要是由于基底后段（P=0.049）和外侧段（P=0.005），整体收缩应变与 LE 量相关（r=0.543；P<0.001）。LE 严重（>2%，n=22）的患者与 LE 轻度（<2%，n=30，-7.1±7.5%）或无 LE（n=49，-16.3±3.3%）的患者相比，在基底后外侧段的变形值最低（-5.9±8.4%）。这些变化伴随着后壁变薄和舒张功能下降，而射血分数和 LV 舒张末期直径没有差异。受试者工作特征分析显示，基底后外侧节段的收缩应变是区分有无 LE 患者的最有力预测因子（敏感度=90%；特异性=97%，曲线下面积=0.913；P<0.001）。
晚期增强与纤维化壁段的纵向应变降低相关。斑点追踪术可作为 FD 中 LE 的间接评估工具。
