基于心脏超声造影的AL型淀粉样变性预后评分系统

Myocardial contrast echocardiography-based prognostic scoring system for primary systemic light chain amyloidosis

目的 寻找与AL型淀粉样变性预后相关的心脏超声造影(myocardial contrast echocardiography,MCE)指标,建立预后评分模型。方法 研究纳入北京大学人民医院2017年11月-2021年4月期间首次诊断为AL型淀粉样变性并进行了MCE检查的39例患者,主要终点是总生存期(overall survival,OS)。使用单因素和多因素Cox回归分析确定独立预测因子及预后模型,通过受试者工作曲线确定各指标的截断值,通过Kaplan-Meier法和log-rank检验比较各组间的预后。结果 在为期713天的中位随访时间后,12例患者死亡。研究选择整体纵向应变(global longitudinal strain,GLS)和室壁运动积分指数(wall motion score index,WMSI)进入OS的预测模型:OS评分=0.18GLS(%)+2.07WMSI(χ^(2)=21.134,P<0.001)。为简化模型,通过受试者工作曲线确认GLS和WMSI的截断值分别为-13.77%和1.16。将GLS>-13.77和WMSI>1.16分别设为1分,39例患者被分为3组(0分组21人,1分组11人,2分组7人);Kaplan-Meier法和log-rank检验提示,0分组的OS最佳,其次分别是1分组和2分组(P<0.001)。结论 MCE检测的GLS、WMSI是AL型淀粉样变性患者OS的独立预测因子,结合GLS和WMSI的模型可以很好地预测AL型淀粉样变性患者的预后。

Objective To explore myocardial contrast echocardiographic(MCE)indicators associated with prognosis in primary systemic light chain amyloidosis(AL amyloidosis)and establish a scoring system to predict the outcome.Methods 39 patients in Peking University People’s Hospital,who were first diagnosed with AL amyloidosis from November 2017 tp April 2021 and underwent MCE examination within 1 month of diagnosis.The primary outcome was the overall survival(OS).The independent predictors were identified using univariate and multivariate Cox analysis.The cut-off points of the scoring systems were determined using receiver operator characteristic curves(ROC curves).The Kaplan–Meier method and the log-rank test were used to compare the prognosis of patients in the different groups.Results After a median follow-up of 713 days,12 deaths occurred.The study selected global longitudinal strain(GLS)and wall motion score index(WMSI)to enter the multivariate analysis model:the OS score=0.18 GLS(%)+2.07 WMSI(chi-square=21.134,P<0.001).To simplify the model,the study used ROC curves to find the cut-off points of GLS and WMSI,which were-13.77 and 1.16,respectively.After the study scored both GLS>-13.77 and WMSI>1.16 as 1 point,39 patients were divided into 3 groups(0-point group,n=21;1-point group,n=11;2-point group,n=7).The Kaplan-Meier method and the log-rank test showed that the 0-point group had the longest OS,followed by the 1-point group,and then the 2-point group(P<0.001).Conclusion GLS and WMSI are the independent MCE risk factors for OS in AL amyloidosis.MCE model including GLS and WMSI can provide additional information to predict the prognosis in AL amyloidosis.