心肌淀粉样变性患者的临床特征及预后分析

目的:分析心肌淀粉样变性患者的临床特征并分析影响患者生存率的因素。方法:针对重庆市部分医院经病理检查确诊的34例心肌淀粉样变性患者进行临床特征统计分析。应用多因素Logistic回归分析寻找可能影响患者生存的因素。结果:34例确诊患者中,从出现症状到明确诊断的时间为(15.2±2.9)个月(1~72个月)。初诊时,有17例(50.0%)误诊,其中8例(23.5%)误诊为肥厚性心肌病,4例(11.8%)误诊为缺血性心肌病,2例(5.9%)误诊为肺原性心脏病,2例(5.9%)误诊为高血压性心脏病,1例(2.9%)误诊为心肌炎。15例(44.1%)患者在确诊时存在心肌损伤心肌肌钙蛋白T(cTnT)≥0.035 ng/ml]。25例(73.5%)患者的超声心动图表现为心室壁不同程度的增厚。15例(44.1%)患者采取了化疗治疗。12例(35.3%)患者在1年内死亡。Logistic回归分析显示,cTnT≥0.035 ng/ml的患者1年内死亡风险高(HR=8.931,95%CI:1.198~66.569,P=0.033)。结论:心肌淀粉样变性的误诊率高,心肌损伤可提示患者1年内死亡风险增高。

Analysis of Clinical Features and Prognosis of Patients With Cardiac Amyloidosis

Objectives:To analyze the clinical features of patients with cardiac amyloidosis and to define determinants related to the survival of these patients.Methods:The clinical characteristics of 34 patients,who hospitalized in hospitals in Chongqing and with complete data and confirmed diagnosis by pathological examinations,were included in this study.Patients were divided into non-survival group and survival group.Multivariate logistic regression analysis was used to define factors which might affect the survival of patients.Results:Duration from the onset of symptoms to a clear diagnosis was(15.2±2.9)months(1-72 months)in these patients.There were 17 misdiagnosis(50%)at initial medical contact,of which 8 cases(23.5%)were misdiagnosed as hypertrophic cardiomyopathy,and 4 cases(11.8%)were misdiagnosed as ischemic cardiomyopathy,2 cases(5.9%)were misdiagnosed as pulmonary heart disease,2 cases(5.9%)were misdiagnosed as hypertensive heart disease and 1 case(2.9%)was misdiagnosed as myocarditis.Evidence of myocardial insult was found in 15 patients(44.1%)(cTnT≥0.035 ng/ml)at the time of diagnosis.In 25 cases(73.5%),echocardiography showed varying degrees of left ventricular hypertrophy.Fifteen patients(44.1%)received chemotherapy treatment.Twelve patients(35.3%)died within 1 year.Logistic analysis showed that increase of cTnT≥0.035 ng/ml was associated with increased risk of death within 1 year(HR=8.931,95%CI:1.198-66.569,P=0.033).Conclusions:The misdiagnosis rate of cardiac amyloidosis is high at initial medical contact and increases cTnT(≥0.035 ng/ml)is associated with increased risk of death within one year in patients with cardiac amyloidosis.