心脏淀粉样变性的临床特征  

Clinical features of cardiac amyloidosis

作　　者：钟诗颖[1] 黄凯[1] 郑静[1] 何红文[1] 郭盛兰[2] 王芬[2] ZHONG Shi-ying;HUANG Kai;ZHENG Jing;HE Hong-wen;GUO Sheng-lan;WANG Fen(CCU,the First Affiliated Hospital of Guangxi Medical University,Nanning 530021,China;Department of Ultrasonic Diagnosis,the First Affiliated Hospital of Guangxi Medical University,Nanning 530021,China)

机构地区：[1]广西医科大学第一附属医院CCU,南宁市530021 [2]广西医科大学第一附属医院超声诊断科,南宁市530021

出　　处：《广西医学》2019年第16期2074-2078,共5页Guangxi Medical Journal

摘　　要：目的 分析心脏淀粉样变性(CA)的临床特征。方法 回顾性分析17例CA患者的临床资料,总结其临床表现、实验室检查及心脏相关检查的特点、诊疗情况,分析误诊原因及治疗效果。结果 所有患者均表现为慢性心力衰竭急性发作,部分患者合并肾脏损害(58.8%)、肝脏损害(17.6%)、多浆膜腔积液(35.3%)。心电图提示肢体导联低电压13例(76.5%),心脏彩超提示有不同程度的多部位增厚15例(88.2%),心脏MRI检查提示有不同程度的延迟强化16例(94.1%)。入院时仅3例(17.6%)疑诊为CA,误诊为肥厚型心肌病、冠心病、高血压性心脏病、缩窄性心包炎各6例(35.3%)、4例(23.5%)、2例(11.8%)、2例(11.8%)。17例CA患者均进行了心外组织病理活检,活检组织均存在淀粉样变沉积。2例(11.8%)诊断为多发性骨髓瘤继发引起的CA,其余15例(88.2%)考虑为原发性免疫球蛋白轻链型CA。随访截止时,12例患者死亡,仅5例存活。结论 CA患者预后差,临床表现多样且缺乏特异性,易误诊,需联合临床表现、心电图、心脏超声心动图及心脏MRI进行诊断,必要时行心内或心外组织活检以确诊。Objective To analyze the clinical characteristics of cardiac amyloidosis(CA).Methods The clinical data of 17 patients with CA were retrospectively analyzed.The clinical manifestations,characteristics of laboratory tests and related cardiac examinations,diagnosis and therapy were summarized,moreover,causes of missed diagnosis and efficacy of therapy were analyzed.Results All of the patients manifested acute attack of chronic heart failure,and parts of the patients were complicated with renal injury(58.8%),liver injury(17.6%) and multiple serous effusion(35.3%).Low voltage in limb leads was observed on electrocardiogram in 13(76.5%) cases,multiple sites thickening of various degrees on echocardiography in 15(88.2%) cases,and delayed enhancement of various degrees on cardiac MRI in 16(94.1%) cases.At admission,only 3(17.6%) cases were suspiciously diagnosed as CA,and 6(35.3%) cases,4(23.5%) cases,2(11.8%) cases and 2(11.8%) cases were misdiagnosed as hypertrophic cardiomyopathy,coronary heart disease,hypertensive heart disease and constrictive pericarditis,respectively.All of the 17 cases received extracardiac pathologic biopsy,and amyloid deposition existed in all of the biopsy tissues.Two(11.8%) cases were diagnosed as CA secondary to multiple myeloma,and the remaining 15(88.2%) cases as primary immunoglobulin light-chain CA.At the end of follow-up,12 cases died and only 5 cases survived.Conclusion CA patients experience poor prognosis,having a variety of clinical manifestations and lacking specificity,and missed diagnosis easily occurs.Diagnosis should be performed in combination with clinical manifestations,electrocardiogram,cardiac echocardiography and cardiac MRI,and it is necessary to perform endomyocardial or extracardiac biopsy for definite diagnosis.

关 键 词：心脏淀粉样变性 临床特征 诊断 误诊 心脏超声检查 心脏核磁共振检查 

分 类 号：R541[医药卫生—心血管疾病]