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心脏磁共振定量评价轻链型心脏淀粉样变患者心肌纤维化的初步研究

郭玉博 王雪竹 李潇 高雅娟 田庄 李剑 霍力 王怡宁

郭玉博, 王雪竹, 李潇, 高雅娟, 田庄, 李剑, 霍力, 王怡宁. 心脏磁共振定量评价轻链型心脏淀粉样变患者心肌纤维化的初步研究[J]. 罕见病研究, 2023, 2(1): 43-49. doi: 10.12376/j.issn.2097-0501.2023.01.006
引用本文: 郭玉博, 王雪竹, 李潇, 高雅娟, 田庄, 李剑, 霍力, 王怡宁. 心脏磁共振定量评价轻链型心脏淀粉样变患者心肌纤维化的初步研究[J]. 罕见病研究, 2023, 2(1): 43-49. doi: 10.12376/j.issn.2097-0501.2023.01.006
GUO Yubo, WANG Xuezhu, LI Xiao, GAO Yajuan, TIAN Zhuang, LI Jian, HUO Li, WANG Yining. Preliminary Study on Quantitative Evaluation of Myocardial Fibrosis by CardiacMagnetic Resonance in Patients with Light Chain Cardiac Amyloidosis[J]. Journal of Rare Diseases, 2023, 2(1): 43-49. doi: 10.12376/j.issn.2097-0501.2023.01.006
Citation: GUO Yubo, WANG Xuezhu, LI Xiao, GAO Yajuan, TIAN Zhuang, LI Jian, HUO Li, WANG Yining. Preliminary Study on Quantitative Evaluation of Myocardial Fibrosis by CardiacMagnetic Resonance in Patients with Light Chain Cardiac Amyloidosis[J]. Journal of Rare Diseases, 2023, 2(1): 43-49. doi: 10.12376/j.issn.2097-0501.2023.01.006

心脏磁共振定量评价轻链型心脏淀粉样变患者心肌纤维化的初步研究

doi: 10.12376/j.issn.2097-0501.2023.01.006
*郭玉博和王雪竹为共同第一作者
详细信息
    通信作者:

    霍力,E-mail:huoli@pumch.cn

    王怡宁,E-mail:wangyining@pumch.cn

  • 中图分类号: R445.2; R542.2+3

Preliminary Study on Quantitative Evaluation of Myocardial Fibrosis by CardiacMagnetic Resonance in Patients with Light Chain Cardiac Amyloidosis

More Information
  • 摘要:   目的  心肌纤维化是轻链型淀粉样变性的潜在致病机制,本研究通过将心脏磁共振(CMR)心肌组织特征和形态、功能参数与68Ga-成纤维细胞激活蛋白抑制剂(FAPI)PET显像相关联,旨在探索CMR定量参数与心肌纤维化过程的相关性。  方法  选取2021年8—12月在北京协和医院确诊的轻链型心脏淀粉样变(AL-CA)患者,进行CMR和68Ga-FAPI PET/CT检查, 登记患者的临床表现及辅助检查等信息,并进行分析。  结果  共纳入23例AL-CA患者,15例(65.2%)为男性,平均年龄(58.3±6.5)岁。具有68Ga-FAPI-04高摄取的患者心肌细胞外容积(ECV)升高,明显高于阴性组患者(P=0.047),且患者的心肌ECV与心肌FAPI摄取呈正相关(r=0.628,P=0.001;r=0.727,P<0.001;r=0.661,P=0.001)。与阴性组患者相比,阳性组患者的左心室(LV)射血分数(EF)(P<0.001)减低。LVEF(r=-0.798,P<0.001;r=-0.794,P<0.001;r=-0.795,P<0.001)、右心室(RV) EF(r=-0.735,P<0.001;r=-0.739,P<0.001;r=-0.684,P<0.001)与心肌FAPI摄取呈负相关,LV周向应变(r=0.668,P<0.001;r=0.708,P<0.001;r=0.705,P<0.001)、LV纵向应变(r=0.629,P=0.001;r=0.635,P=0.001;r=0.597,P=0.003)、RV纵向应变(r=0.575,P=0.004;r=0.792,P<0.001;r=0.673,P<0.001)与心肌FAPI摄取呈负相关。  结论  FAPI反映的成纤维细胞活化伴有CMR反映的间质特征异常及其导致的心肌运动功能减低。FAPI摄取增高的AL-CA患者的ECV升高,EF减低,应力减低并形态学异常。

     

  • 图  1  AL-CA患者的CMR图像

    A.FAPI摄取阳性患者的CMR图像(延迟强化,T1 mapping,ECV,左心室径向、周向及纵向应变); B.FAPI摄取阴性患者的CMR图像(延迟强化,T1 mapping,ECV,左心室径向、周向及纵向应变)

    Figure  1.  CMR in patients with AL-CA

    图  2  AL-CA患者CMR参数与PET/CT参数相关性

    Figure  2.  The correlation between CMR parameters and PET/CT parameters in AL-CA patients

    表  1  轻链型心脏淀粉样变(AL-CA)患者临床特征及心脏磁共振(CMR)参数比较

    Table  1.   The clinical features and cardial magnetic resonance(CMR) parameters of the light chain cardiac amyloidosis(AL-CA) patients

    指标 数值(N=23) 68Ga-FAPI-04摄取 P
    阳性组(n=17) 阴性组(n=6)
    男性(例, %) 15 (65.2) 13 (76.5) 2 (33.3) 0.001
    年龄(岁) 58.3±6.5 59.2±6.4 55.7±6.8 0.639
    NYHA分级≥ Ⅱ(例, %) 17 (73.9) 17 (100.0) 0 (0) <0.001
    梅奥分期≥ Ⅱ(例, %) 17 (73.9) 16 (94.1) 1 (16.7) <0.001
    dFLC(mg/L) 251.8 (97.5,451.2) 344.6 (250.6,487.7) 55.3 (29.8,107.6) <0.001
    肌钙蛋白Ⅰ(μg/L) 0.053 (0.017,0.133) 0.110 (0.028,0.162) 0.017 (0.017,0.017) 0.004
    NT-proBNP(pg/mL) 2606 (191,8520) 3931 (2185,10 475) 138.5 (51.5,184.3) <0.001
    native T1(ms) 1398.0±112.2 1415.2±124.8 1345.7±35.8 0.055
    ECV 47.7±14.0 53.1±12.3 32.5±2.9 0.047
    T2(ms) 44.5±4.3 45.6±3.7 41.7±5.0 0.149
    LVEDVi(mL/m2) 71.9±13.7 73.3±13.7 68.0±14.0 0.920
    LVESVi(mL/m2) 30.8 (22.5,39.0) 32.9 (29.5,40.3) 19.3 (17.2,25.9) <0.001
    LVEF(%) 57.5 (46.4,67.0) 52.0 (40.7,59.0) 70.9 (66.5,71.3) <0.001
    LVMi(g/m2) 76.3±25.9 85.5±23.4 50.0±9.2 0.067
    左心房容积(cm2) 69.4±26.5 74.9±26.3 54.0±22.1 0.668
    RVEDVi(mL/m2) 73.1±18.1 77.3±18.3 61.3±11.8 0.390
    RVESVi(mL/m2) 35.3 (23.6,48.1) 41.1 (28.3,49.3) 23.1 (15.6,25.9) 0.002
    RVEF(%) 51.0±14.7 46.3±14.0 64.4±5.5 0.112
    RVMi(g/m2) 19.9 (14.7,26.9) 22.5 (16.7,29.3) 13.9 (10.2,15.1) 0.002
    右心房容积(cm2) 73.8±26.8 82.8±24.8 48.7±13.3 0.075
    LV周向应变(%) -17.3±4.93 -15.5±4.5 -22.3±1.6 0.168
    LV纵向应变(%) -9.5±5.0 -7.6±4.2 -14.9±2.2 0.051
    LV径向应变(%) 27.1±11.1 23.0±9,2 38.9±6.5 0.644
    RV周向应变(%) -8.2 (-13.2,-5.6) -7.8 (-13.3,-5.9) -9.4 (-12.9,7.9) 0.973
    RV纵向应变(%) -8.0±6.7 -5.5±5.8 -15.2±3.1 0.399
    RV径向应变(%) 45.2 (25.3,71.6) 35.4 (21.1,78.4) 60.5 (54.9,76.1) 0.062
    下载: 导出CSV

    表  2  AL-CA患者生物标志物、CMR参数与PET/CT参数相关性分析

    Table  2.   The correlation between serum biomarkers, CMR parameters, and PET/CT parameters in AL-CA patients

    指标 SUVmean SUVmax SUVR
    r P r P r P
    dFLC(mg/L) 0.607 0.002 0.603 0.002 0.541 0.008
    肌钙蛋白I(μg/L) 0.386 0.069 0.336 0.117 0.366 0.086
    NT-proBNP(pg/mL) 0.768 <0.001 0.722 <0.001 0.717 <0.001
    native T1(ms) 0.153 0.486 0.166 0.449 0.093 0.673
    ECV 0.628 0.001 0.727 <0.001 0.661 0.001
    T2(ms) 0.282 0.193 0.277 0.200 0.157 0.474
    LVEDVi(mL/m2) 0.260 0.231 0.271 0.211 0.231 0.288
    LVESVi(mL/m2) 0.829 <0.001 0.811 <0.001 0.785 <0.001
    LVEF(%) -0.798 <0.001 -0.794 <0.001 -0.795 <0.001
    LVMi(g/m2) 0.524 0.010 0.620 0.002 0.602 0.002
    左心房容积(cm2) 0.652 0.001 0.587 0.003 0.504 0.014
    RVEDVi(mL/m2) 0.592 0.003 0.474 0.022 0.474 0.022
    RVESVi(mL/m2) 0.725 <0.001 0.675 <0.001 0.626 0.001
    RVEF(%) -0.735 <0.001 -0.739 <0.001 -0.684 <0.001
    RVMi(g/m2) 0.664 0.001 0.583 0.004 0.470 0.024
    右心房容积(cm2) 0.687 <0.001 0.661 0.001 0.615 0.002
    LV周向应变(%) 0.668 <0.001 0.708 <0.001 0.705 <0.001
    LV纵向应变(%) 0.629 0.001 0.635 0.001 0.597 0.003
    LV径向应变(%) -0.480 0.020 -0.488 0.018 -0.535 0.009
    RV周向应变(%) 0.215 0.323 0.153 0.485 -0.002 0.993
    RV纵向应变(%) 0.575 0.004 0.792 <0.001 0.673 <0.001
    RV径向应变(%) -0.447 0.033 -0.358 0.094 -0.272 0.210
    下载: 导出CSV
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  • 收稿日期:  2022-11-22
  • 修回日期:  2022-12-02
  • 网络出版日期:  2023-03-07

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