国人心肌淀粉样变性Meta分析  

Meta analysis of Chinese cardiac amyloidosis

作　　者：伍崇海 黄桢钧 徐丽敏[1] 钟赟[1] 王静[1] 付大伟[1] 徐如芹 叶晓兰[1] 刘世明[1] 

机构地区：[1]广州医科大学附属第二医院心内科广州心血管疾病研究所,广州510260 [2]广州医科大学

出　　处：《临床心血管病杂志》2014年第7期622-625,共4页Journal of Clinical Cardiology

摘　　要：目的：分析国人心肌淀粉样变性（cardiac amyloidosis,CA）的临床特点及探讨早期诊断CA的依据和方法。方法：检索中国期刊网全文数据库、万方数据库,收集2013-06-30前发表的病例分析和病例报告,并进行回顾性分析。结果：①经检索共纳入文献83篇计429例。②CA男性发病率高于女性（2.87∶1）,发病年龄（55.72±6.52）岁。③临床表现以心力衰竭为主（Ⅲ~Ⅳ级占71.2%）,最常见的主诉症状为双下肢水肿和气促。其他常见临床表现：心包积液,蛋白尿。④CA在心电图、超声心动图、心脏磁共振中有特征性表现。⑤临床中CA易误诊为肥厚型心肌病。⑥CA尚无特效治疗,预后差。结论：CA临床表现多样化,易误诊,难治性/进行性/顽固性心力衰竭＋多浆膜腔积液＋蛋白尿可作为怀疑CA的依据之一,心肌病理活检为确诊方法,但开展难度大,而＂质/电矛盾＂和室间隔的厚度〉1.98cm、RⅠ电压/左心室后壁〈0.4、RV5（6）电压/左心室后壁〈0.7、＂闪耀征＂易开展且具科学性,为CA的早期诊断提供依据,心脏磁共振和心肌外活检也可作为进一步确诊的依据。Objective：To analyze the clinical characteristics of Chinese cardiac amyloidosis and to investigate the meth- od for early diagnosis of cardiac amyloidosis. ＇Method.. Literatures on cardiac amyloidosis published in journals before June 30, 2013 were identified by searching CNKI and Wanfang, and retrospective analysis was performed. Result：（1）A total of 83 literatures with 429 cardiac amyloidosis patients were found. （2）The male incidence was evidently higher than female （2.87 ： 1）. Mean age of these patients was （55.72±6.52） years old. （3）The primary clinical manifestation was heart failure, especially the NYHA class Ⅲ and Ⅳ heart failure accounted for 71.2%. With the most common Chief Complaint.. such as edema of both lower extremities anddyspnea. And other common clinical manifestations included pericardial effu- sion and proteinuria. （4）Cardiac amyloidosis could be characteristic in electrocardiograph（ECG）, echocardiogram and cardi- ac magnetic resonance imaging. （5）Cardiac amyloidosis was oftenmisdiagnosed as hypertrophic cardiomyopathy. （6） There was no specific treatment for cardiac amyloidosis, and the prognosis was poor. Conclusion.. The clinical manifestations of cardiac amyloidosis are multiple and easily misdiagnosed. Refractory/progressive/intractable heart failure and multiple mucous membranes effusion and proteinuria can be used as one of the suspected cardiac amyloidosis evidences. Endomyocardial biopsy is the final diagnostic method, but it is difficult to be carried out. However, if a low voltage was present and the interventricular septal thickness is 〉1.98cm, ratio of RI voltage / left ventricular posterior wall （ 0. 4, ratios of RV5 （ 6 ） voltage / left ventricular posterior wall 〈 0. 7and ＂sparkling appearance＂ are easily to be carried out and scientific, which can provide evidences for early diagnosis. Cardiac amyloidosis can also get further diagnosis, in light of cardiac magnetic resonance imaging and unendomyocardial biopsy.

关 键 词：心肌疾病 心肌淀粉样变性 临床特点 

分 类 号：R542.2[医药卫生—心血管疾病]