淀粉样变性心肌病268例临床分析

目的 总结淀粉样变性心肌病(cardiac amyloidosis,CA)的临床特点、诊治方法,为临床进一步认识和诊治该病提供依据.方法 检索中国期刊全文数据库(CNKI)、西文生物医学文献数据库、万方数据库和pubmed,收集2000年1月至2009年10月发表的相关病例报告,进行回顾性调查研究.结果 ①经检索共筛选出相关文献44篇计268例;②CA可发生于任何年龄,多见于中老年,男性发病率明显高于女性(2.67∶1);③临床表现最常见的是进行性的心力衰竭(67.1%),其中双下肢水肿(54.3%),气短(44.5%),肝大(39.0%),颈静脉怒张(34.8%);其次为类似心绞痛的症状--胸闷胸痛(25.6%),心血管系统以外最常受累的是肾脏(37.2%);④临床上易被误诊,其中58.9%误诊为肥厚性心肌病,28.8%误诊为限制性心肌病.⑤心肌病理活检为确诊方法,心电图、心脏彩色超声和磁共振是诊断CA主要的辅助检查,其中磁共振有望成为一种新的无创确诊方法.⑥CA患者合并心力衰竭多预后不良.结论 CA临床表现多样化,易误诊为肥厚性心肌病和限制性心肌病,且合并心力衰竭时多预后不良,应及早发现与诊治.

Clinical analysis on 268 cases of cardiac amyloidosis: a literature review (2000 to 2009 )

Objective To comprehend the clinical characteristics and treatment of present status of cardiac amyloidosis.Methods Literatures on cardiac amyloidosis published in journals between January 2000 and October 2009 were identified by searching CNKI,Wanfang,pubmed and FMJS.Retrospective review was done.Results ①A total of 44 literatures with 268 cardiac amyloidosis patients were found.②Cardiac amyloidosis occurred in any age,especially the middle or elder people.The male incidence was obviously higher than female with the rate of 2.67∶1.③The most common clinical manifestation was progressively congestive heart failure（67.1%）,such as edema of both lower extremities（54.3%）,dyspnea（44.5%）,hepatomegaly（39.0%） and distention of jugular vein（34.8%）.The other common symptom was chest pain（25.6%）.Out of cardiovascular system,kidney（37.2%） was the most affected organ.④Cardiac amyloidosis was fallibility misdiagnosed multiple diseases,such as hypertrophic cardiomyopathy（58.9%） and restrictive cardiomyopathy（28.8%）.⑤Endomyocardial biopsy was the final diagnostic method.Electrocardiogram,echocardiogram and cardiovascular magnetic resonance imaging（CMRI） were important auxiliary examinations.It is possible that CMRI becomes a new noninvasive diagnostic method.⑥The cases with congestive heart failure had a poor prognosis.Conclusion The clinical characteristics of cardiac amyloidosis are multiple,can be fallibility misdiagnosed,such as hypertrophic cardiomyopathy and restrictive cardiomyopathy.Cardiac amyloidosis has a poor prognosis,especially with congestive heart failure.It is essential to diagnose and treat as early as possible.