Lehmonen Lauri, Kaasalainen Touko, Atula Sari, Mustonen Tuuli, Holmström Miia
HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, Haartmaninkatu 4, PO Box 340, 00029 HUS, Helsinki, Finland.
Department of Physics, University of Helsinki, Helsinki, Finland.
Int J Cardiovasc Imaging. 2019 Feb;35(2):351-358. doi: 10.1007/s10554-019-01570-4. Epub 2019 Mar 8.
Gelsolin (AGel) amyloidosis is a hereditary condition with common neurological effects. Myocardial involvement, especially strain, T1, or extracellular volume (ECV), in this disease has not been investigated before. Local myocardial effects and possible amyloid accumulation were the targets of interest in this study. Fifty patients with AGel amyloidosis were enrolled in the study. All patients underwent cardiovascular magnetic resonance imaging, including cine imaging, T1 mapping, tagging, and late gadolinium enhancement (LGE) imaging at 1.5 T. Results for volumetry, myocardial feature-tracking strain, rotation, torsion, native T1, ECV, and LGE were investigated. The population mean native T1 values in different segments of the left ventricle (LV) varied between 1003 and 1080 ms. Myocardial mean T1 was 1031 ± 37 ms. T1 was highest in the basal plane of the LV (1055 ± 40 ms), similarly to ECV (30.0% ± 4.4%). ECV correlated with native T1 in all LV segments (p < 0.005). Basal LGE was detected in 76% of patients, and mid-ventricular LGE in 32%. LV longitudinal strain was impaired (- 17.4% ± 2.6%), significantly decreasing apical rotation (p = 0.018) and concurrently myocardial torsion (p = 0.005). LV longitudinal strain correlated with mean T1 and ECV of different LV planes (p < 0.04; basal p < 0.01). Myocardial involvement in AGel amyloidosis is significant, but the effects are local, focusing on the basal plane of the LV.
凝溶胶蛋白（AGel）淀粉样变性是一种具有常见神经学影响的遗传性疾病。此前尚未对该疾病中的心肌受累情况，尤其是应变、T1或细胞外容积（ECV）进行过研究。本研究的关注点是局部心肌效应和可能的淀粉样蛋白积聚。50例AGel淀粉样变性患者纳入本研究。所有患者均接受了心血管磁共振成像检查，包括在1.5T场强下的电影成像、T1 mapping、标记成像和延迟钆增强（LGE）成像。研究了容积分析、心肌特征追踪应变、旋转、扭转、固有T1、ECV和LGE的结果。左心室（LV）不同节段的总体平均固有T1值在1003至1080毫秒之间。心肌平均T1为1031±37毫秒。T1在LV基底部平面最高（1055±40毫秒），与ECV情况类似（30.0%±4.4%）。在所有LV节段中，ECV与固有T1相关（p<0.005）。76%的患者检测到基底LGE，32%的患者检测到心室中部LGE。LV纵向应变受损（-17.4%±2.6%），显著降低心尖旋转（p=0.018）并同时降低心肌扭转（p=0.005）。LV纵向应变与不同LV平面的平均T1和ECV相关（p<0.04；基底p<0.01）。AGel淀粉样变性中的心肌受累较为显著，但影响是局部的，主要集中在LV的基底部平面。
