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

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

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^(68)Ga-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^(68)Ga-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^(68)Ga-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。

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 thefunctional parameters with gallium-68-labeledfibroblast activation protein inhibitor 04 positron emission tomography(^(68)Ga-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^(68)Ga-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^(68)Ga-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.