| Background and objectiveCardiac amyloidosis is a rare disease,whose clinical symptoms are atypical.Clinicians have insufficient understanding of cardiac amyloidosis.Therefore,the early diagnostic rates are low and misdiagnostic rates are high.In addition,the overall prognosis of cardiac amyloidosis remains poor.At present^serum biomarkers are mainly used for risk stratification in AL-CA;but when there is hepatic and kidney insufficiency,the validity of the current staging system will decline,and more effective non-invasive methods are needed to assess the prognosis of patients.There are two purposes of this study:①to summarize the clinical characteristics of primary light-chain cardiac amyloidsis confirmed by the First Affiliated Hospital of Zhengzhou University from 2015 to 2021;②to analyze the risk factors affecting the survival of primary light cardiac amyloidsis in this study.Methods1.Collected clinical datas of 72 patients with primary light-chain cardiac amyloidosis treated at the First Affiliated Hospital of Zhengzhou University from January 2015 to October 2021,including general clinical data,laboratory examination indicators,echocardiographic parameters,and strengthening methods,range and related parameters of late gadolinium enhancement in cardiac magnetic resonance.The clinical features of patients with cardiac amyloidosis were concluded.2.The end point event was the full cause of death.After an average 16-month follow-up,36 out of the 72 patients died.Patients was divided into survival groups and death groups.The SPSS 19.0 statistical software was used in this study.The differences between death and survival groups in sex,age,chemotherapy,serum free light chain,B-type natriuretic peptide,Caridac troponin,left ventricular stroke volume,left ventricular mass index,late gadolinium enhancement range,were compared by student’s t test,nonparameter Mann-Whitney U test,Chi-square test and Fisher exact probability method according the variable type、normality and homogeneity of variance.The possible independent risk factors of prognosis with regarding to primary light-chain cardiac amyloid patients were analyzed by using univariate and multivariate Cox proportional hazard modeling.The Kaplan-Meier survival analysis was applied to compare the survival time differences of the risk factors affecting the prognosis across the different groups,and the survival curves were plotted.Results1.In the 72 patients of this study,the number of men accounted for 65.3%;the median onset age was late,56 years;the most common onset symptom was the edema of lower extremity,and the time form onset to specific diagnosis was 5 months;the highest positive rate of biopsy was the kidney,100%;the positive rate of biopsy was higher in organ biopsy than tissue biopsy;kidney was the most common accompanied organ,accounting for 86.1%;liver involved was only 9.2%.λ type was the main light chain type,accounting for 65.3%.N-terminal B-type natriuretic peptide,and cardiac troponin Ⅰ and the difference between involved and uninvolved serum free light chain were all significantly higher than normal value.The patients at Mayo and BU clinical stage Ⅲ accounted for 64.8%.The overall median survival time was short,at only 13.5months.The most common occurrence in electrocardiogram was arrhythmia(58.3%),there were low occurrence in low voltage of limb leads,poor chest R wave increasing and pseudopathological Q wave,with 45.8%,25.0%and 23.6%,respectively.Ventricular wall thickening occurred in 86.1%of patients on echocardiography,with an overall higher mean LV ejection fraction of 58.8%and 64(88.9%)of patients with LV ejection fraction>50%.19 patients(26.4%)showed thickening and enhancement of myocardial echo and granular sensation,clearly indicating cardiac amyloidosis.All of the 31 patients who underwent CMR LGE presented 30 patients with varying degrees of subendocardial or transmural enhancement.2.The variants of death and survival group were compared.We found that,compared the survival group,the time from the onset of symptoms to specific diagnosis was longer.And the median survival time and the number of cases receiving chemotherapy in the death group were lower than survival group.Compared with the survival group,left ventricular end-diastolic volume and stroke volume were lower,the ventricular interval thickness was thicker,and the left ventricular mass index was higher,which all had statistically significance.3.The parameters that may affect the prognosis of primary light chain cardiac amyloidosis were analysed by univariate Cox proportional hazard modeling.Then we found that without receiving chemotherapy,presence of pseudo-pathological Q waves,intraventricular septum thickness,stroke volume might affect patient’s survival.The ROC curve was applied to determine the cut-off value of stroke volume as 56.5ml.And further using multi-variate Cox proportional hazard modeling analysis found that without receiving chemotherapy(HR 2.885,P=0.004),presence of pseudo-pathological Q waves(HR 2.245,P=0.033)and stroke volume<56.5ml(HR 2.624,P=0.014)were risk factors affecting survival.4.According the cut-off value of stroke volume as 56.5ml,dividing patients into high stroke volume group(≥56.5ml)and low stroke volume group(<56.5ml).Survival analysis by Kaplan-Meier found that the median survival time of patients without receiving chemotherapy,presence of pseudo-pathological Q wave,and low stroke volume group was shorter than that of patients receiving chemotherapy,without pseudo-pathological Q wave,and high stroke volume group,41 to 7 months,38 to 17months and 41 to 20months,respectively,with statistically significant differences.Conclusions1.Primary light-chain cardiac amyloidosis is more common in men,with later onset age.Kidney involvement is more common accompanied.There is higher positive rate in organ biopsy compared with tissue biopsy.The overall median survival time is short,only 13.5 months.2.The clinical manifestations of heart failure such as lower extremity edema and chest tightness are the most common onset symptoms of AL-CA.Cardiac magnetic resonance has extremely high diagnostic value.3.Without receiving chemotherapy、presence of pseudo-pathological Q wave、decreased stroke volume are associated with all-cause death in patients with primary light-chain cardiac amyloidosis. |
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