首页|39例轻链型心脏淀粉样变患者的临床特征

39例轻链型心脏淀粉样变患者的临床特征

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目的:分析39例轻链型心脏淀粉样变(light chain cardiac amyloidosis,AL-CA)患者的临床特征,寻找可能对患者生存率产生影响的因素.方法:对2018年12月至2023年12月期间在某院住院,经病理确诊的39例AL-CA患者依据临床特征分为1年内死亡组和存活组,分析影响患者1年内生存率的可能因素.结果:39例纳入研究的患者中,常规心电图结果以肢导联QRS低电压、窦性心律不齐等为主;超声心动图提示左室壁对称性增厚,二尖瓣反流(中重度),左室收缩及舒张功能减低;其中17例患者行心脏磁共振(CMR)检查,结果均提示左室壁及室间隔明显增厚、心室腔狭窄、双房增大,符合心脏淀粉样变改变.检验结果提示:除2例患者的cTnT结果正常外,其余患者的cTnT结果均增高;NT-proBNP结果均增高.运用二元Logistic回归分析发现,cTnT升高可提示患者1年内死亡风险增大.病理活检结果提示:所有活检组织光镜检查经刚果红染色呈砖红色.结论:轻链型心脏淀粉样变临床症状不典型,易误诊,当患者出现心力衰竭表现伴心包积液,且心电图表现为肢体导联低电压,超声心动图提示心室壁增厚,NT-proBNP和cTnT血浆浓度增高,病理活检刚果红染色呈砖红色有助于明确心脏淀粉样变的临床诊断.心肌损伤患者1年内死亡风险增高.
Clinical characteristics analysis of 39 patients with light chain cardiac amyloidosis
Objective:To analyze the clinical characteristics of 39 patients with light chain cardiac amyloidosis and identify factors that may affect their survival rate.Methods:39 patients with mild chain amyloidosis diagnosed by pathology and admitted to our hospital from December 2018 to December 2023 were divided into a 1-year death group and a survival group according to clinical characteristics.The differences between the two groups were analyzed to identify factors that may affect the 1-year survival rate of patients.Results:Among the 39 patients included in the study,routine electrocardiogram results mainly showed symptoms such as limb lead QRS low voltage and sinus arrhythmia;Echocardiography suggests symmetrical thickening of the left ventricular wall,mitral regurgitation(moderate to severe),and reduced left ventricular systolic and diastolic function;Among them,17 patients underwent cardiac magnetic resonance(CMR)examination,and the results showed significant thickening of the left ventricular wall and interventricular septum,narrowing of the ventricular cavity,and enlargement of both atria,consistent with changes in cardiac amyloidosis.The test results indicate that except for 2 patients with normal cTnT results,the cTnT results of the remaining patients have increased to varying degrees;The results of NT proBNP were all increased.Using binary logistic regression analysis,it was found that an increase in cTnT can indicate an increased risk of death within one year for patients.Pathological biopsy results indicate that all biopsy tissues were stained with Congo red under pathological light microscopy and appeared brick red.Conclusion:The clinical symptoms of light chain cardiac amyloidosis are atypical and prone to misdiagnosis.When patients present with heart failure,pericardial effusion is present,and the electrocardiogram shows low voltage in limb leads,echocardiography suggests thickening of the ventricular wall,blood sample examination shows increased concentrations of NT proBNP and cTnT,and pathological biopsy shows brick red staining,which helps to clarify the clinical diagnosis of cardiac amyloidosis.Patients with myocardial injury have an increased risk of death within one year.

light chain cardiac amyloidosisclinical featuresimmunofixation electrophoresispathological biopsy

赵文心、张高丽、邓予晖、张珂、赵美晨、于丽霞、许泼实

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河南省人民医院/阜外华中心血管病医院/郑州大学人民医院检验科,河南郑州 451464

轻链型心脏淀粉样变 临床特征 免疫固定电泳 病理活检

2024

河南大学学报(医学版)
河南大学

河南大学学报(医学版)

影响因子:0.494
ISSN:1672-7606
年,卷(期):2024.43(6)