心肌淀粉样变性临床特点及远期预后的影响因素

Clinical features and factors affecting long-term prognosis of patients with cardiac amyloidosis

摘要:

目的 探讨心肌淀粉样变性患者的临床特点及其远期预后的影响因素。方法 回顾性分析119例临床确诊心肌淀粉样变性患者的临床资料,电话随访患者的生存状态,分析患者的临床表现、心电图、心脏超声及心脏磁共振特点及其与远期预后的关系,主要观测终点为全因死亡。应用SPSS 17.0统计软件进行数据分析。结果 119例患者首发症状多样,以气短喘憋和胸闷为主,其次为下肢水肿和乏力,心功能多为美国纽约心脏病学会心功能分级 Ⅲ~Ⅳ级。患者1年生存率50%,5年生存率仅为25%,全因死亡率的独立影响因素包括脑利钠肽前体(NT-proBNP)、肌钙蛋白T、糖抗原125(CA125)、白蛋白、血氯、免疫球蛋白M水平。结论 心肌淀粉样变性患者预后差,1年生存率仅为50%。患者全因死亡率的独立影响因素包括NT-proBNP、肌钙蛋白T、CA125、白蛋白、血氯、免疫球蛋白M水平。

Abstract:

Objective To investigate the clinical features of patients with cardiac amyloidosis and the factors affecting their long-term prognosis. Methods A retrospective study was conducted of 119 patients with clinically diagnosed cardiac amyloidosis by reviewing their clinical data, and survival obtained from follow-up telephone. Analysis was made of their clinical manifestations, electrocardiogram, echocardiogram and cardiovascular magnetic resonance and of their relationship with long-term prognosis, the primary end-point being all-cause mortality. SPSS statistics 17.0 was employed for data analysis. Results Symptoms of the 119 patients varied widely, featuring mainly shortness of breath and chest tightness followed by edema of lower limbs and fatigue. Cardiac function was mostly New York Heart Association (NYHA) Ⅲ-Ⅳ class. One-year mortality rate was 50%, and five-year mortality rate was 25%. Independent factors affecting all-cause deaths included N-terminal pro-brain natriuretic peptide (NT-proBNP), troponin T, carbohydrate antigen 125 (CA125), albumin, blood chloride, and immunoglobulin M (IgM). Conclusion Prognosis of cardiac amyloidosis is poor with a 1-year survival rate of only 50%. Independent factors affecting all-cause deaths include NT-proBNP, troponin T, CA125, serum albumin, blood chloride, and serum IgM.