Predictive Value of Baseline Extracellular Volume for Therapeutic Cardiac Response in Light Chain Cardiac Amyloidosis
10.3969/j.issn.1000-3614.2025.06.005
- VernacularTitle:基线细胞外容积对免疫球蛋白轻链型淀粉样变心肌病治疗后心脏缓解的预测价值
- Author:
Yang LU
1
;
Jingyi LI
;
Yubo GUO
;
Yining WANG
;
Jian LI
;
Zhuang TIAN
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院 心内科,北京 100730
- Publication Type:Journal Article
- Keywords:
light chain cardiac amyloidosis;
cardiac magnetic resonance;
extracellular volume fraction;
cardiac response
- From:
Chinese Circulation Journal
2025;40(6):583-590
- CountryChina
- Language:Chinese
-
Abstract:
Objectives:This study aims to explore the value of the baseline extracellular volume(ECV)measured by cardiac magnetic resonance(CMR)in predicting cardiac response in patients with light chain cardiac amyloidosis(AL-CA)after treatment.Methods:This single-center retrospective cohort study included AL-CA patients diagnosed between May 2020 and March 2023.Baseline ECV measurement and other relevant parameters were derived from CMR.Therapeutic cardiac response was assessed through serial measurements of N-terminal pro-B-type natriuretic peptide(NT-proBNP).Complete recovery was defined as achieving NT-proBNP≤350 pg/ml post-treatment.Patients demonstrating>60%reduction from baseline NT-proBNP without attaining complete response criteria were classified as very good partial recovery.Those showing 31%-60%decreases from baseline NT-proBNP without meeting the threshold for very good partial recovery were qualified as partial recovery,while≤30%reductions from baseline were considered as non-recovery.The study evaluated two endpoints:the initial emergence of any cardiac recovery(encompassing partial recovery,very good partial recovery,or complete recovery)and the subsequent attainment of optimal cardiac recovery(encompassing partial recovery,very good partial recovery,or complete recovery).The patients were divided into two groups based on whether they experienced cardiac recovery at the end of follow-up:the recovery group(n=24,comprising 7 with partial recovery,14 with very good partial recovery,and 3 with complete recovery)and the non-recovery group(n=16).Cox Proportional hazards regression models were used to analyse the impact of baseline ECV on the cardiac recovery.The Kaplan-Meier method and log-rank test were used to assess and compare the probability and timing of cardiac recovery between different baseline ECV groups.Results:Among the 40 patients,28(70%)were male,with a mean age of(58?±?8)years.32 patients(80%)had the λ subtype of AL-CA.During a median follow-up of 568(155,1 049)days,15 patients showed partial cardiac recovery at 60 days post-treatment,and 3 patients achieved very good partial cardiac recovery;by 720 days of treatment and until the end of follow-up,3 patients achieved complete cardiac recovery.Multivariate Cox regression analysis revealed that baseline ECV(HR=0.937,95%CI:0.879-0.999,P=0.045)and daratumumab-based regimens(HR=3.279,95%CI:1.098-9.796,P=0.033)were significant predictors of the initial cardiac recovery.Similarly,baseline ECV(HR=0.931,95%CI:0.867-1.000,P=0.048)and daratumumab-based regimens(HR=3.132,95%CI:1.052-9.319,P=0.040)were also independent predictors for the best cardiac recovery.Kaplan-Meier analysis demonstrated that patients with baseline ECV<54%achieved an earlier first cardiac recovery than those with baseline ECV≥54%(log-rank P=0.014)and the group with baseline ECV<55%were more likely to achieve the best cardiac recovery compared to those with baseline ECV≥55%(log-rank P=0.006).Conclusions:Baseline ECV measured by CMR can serve as an independent predictor of cardiac recovery in AL-CA patients after treatment.Lower baseline ECV levels are associated with a faster and more favorable cardiac recovery.The daratumumab-based regimens demonstrated superior cardiac recovery outcomes.
