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

GUO Yubo; WANG Xuezhu; LI Xiao; GAO Yajuan; TIAN Zhuang; LI Jian; HUO Li; WANG Yining

doi:10.12376/j.issn.2097-0501.2023.01.006

Journal of Rare Diseases > 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

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

GUO Yubo^{1, *},
WANG Xuezhu^{2, *},
LI Xiao¹,
GAO Yajuan³,
TIAN Zhuang^{4, 5},
LI Jian³,
HUO Li^2, ,,
WANG Yining^1, ,

1.
Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College, Beijing 100730, China
2.
Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College, Beijing 100730, China
3.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College, Beijing 100730, China
4.
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College, Beijing 100730, China
5.
Department of International Medical Services, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College, Beijing 100730, China

More Information

Corresponding author:
HUO Li, E-mail: huoli@pumch.cn

WANG Yining, E-mail: wangyining@pumch.cn
Received Date: November 21, 2022
Revised Date: December 01, 2022
Available Online: March 06, 2023

Graphical Abstract

Abstract

Abstract

Objective
Myocardial fibrosis is a potential mechanism of light-chain myocardial amyloidosis(AL-CA). This research aimed at exploring the correlation between multiparameter cardiac magnetic resonance (CMR) and myocardial fibrosis by relating the CMR myocardial tissue characteristics, the morphological and the functional parameters with gallium-68-labeledfibroblast activation protein inhibitor 04 positron emission tomography (⁶⁸Ga-FAPI PET).
Methods
We gave the patients diagnosed with AL-CA in Peking Union Medical College Hospital from August to December 2021 the examinations of CMR and ⁶⁸Ga-FAPI PET/CT. We recorded and analyzed the information on clinical manifestations and examinations of the patients.
Results
A total of 23 patients with AL-CA were included, 15 (65.2%)of which were male and the mean age was 58.3±6.5 years. Patients with high ⁶⁸Ga-FAPI-04 uptake had shown growth in myocardial extracellular volume (ECV), significantly higher than those in the negative group (P=0.047). In addition, patients' myocardial ECV was positively correlated with myocardial FAPI uptake (r=0.628, P=0.001;r=0.727, P < 0.001;r=0.661, P=0.001). Patients in the positive group showd reduced left ventricular (LV) ejection fraction (EF)(P < 0.001).LVEF (r=-0.798, P < 0.001;r=-0.794, P < 0.001; r=-0.795, P < 0.001) and right ventricular (RV)EF (r=-0.735, P < 0.001;r=-0.739, P < 0.001;r=- 0.684, P < 0.001) showd negatively correlated with myocardial FAPI uptake, LV circumferential strain (r=0.668, P < 0.001;r=0.708, P < 0.001;r=0.705, P < 0.001), LV longitudinal strain (r=0.629, P=0.001;r=0.635, P=0.001; r=0.597, P=0.003), and RV longitudinal strain (r=0.575, P=0.004; r=0.792, P < 0.001;r=0.673, P < 0.001) were negatively correlated with myocardial FAPI uptake.
Conclusions
FAPI-related fibroblast activation is concurrent with CMR-related abnormal myocardial interstitial characteristics that leads to the decreased function of the myocardial movement. Patients with increased FAPI uptake present with increased ECV, decreased EF, and decreased strain with morphological abnormalities.

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

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