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

SUN Yu-tong; LIU Yang; HOU Chang; JIN Wen-ying; GAO Si-qi; LU Jin; ZHU Tian-gang; LIU Jian

doi:10.3969/j.issn.1672-5301.2024.09.002

Abstract：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.