42例心肌淀粉样变性的临床特点分析  

Clinical characteristics of 42 patients with cardiac amyloidosis

作　　者：黄雨晴[1] 詹嘉欣[1] 魏学标[1] 陈纪言[1] 周颖玲[1] 蒋磊[1] 冯颖青[1] 

机构地区：[1]广东省人民医院(广东省医学科学院)广东省心血管研究所心内科, 广州510080

出　　处：《中华内科杂志》2014年第7期546-549,共4页Chinese Journal of Internal Medicine

摘　　要：目的 分析和总结心肌淀粉样变性患者的临床特点以提高临床诊疗水平.方法 回顾性分析我院自2008年以来行腹部、心内膜等病理活检确诊的42例心肌淀粉样变性患者的临床表现、心电图、超声心动图特点.结果 心肌淀粉样变性临床表现多样：以胸闷并气促（37例,88.1％）、胸痛（20例,47.6％）、右心衰竭（27例,64.3％）、乏力（27例,64.3％）、肾功能不全和蛋白尿（30例,71.4％）等表现多见;心电图（ECG）：以肢体导联低电压（32例,76.2％）、胸导联R波递增不良（29例,69.0％）、ST-T改变（17例,40.5％）、假性坏死Q波（28例,66.7％）及各种心律失常（36例,85.7％）多见;心脏彩色超声检查：42例（100％）均存在不同程度左心室后壁或室间隔增厚、心肌颗粒样闪光回声增强或毛玻璃样改变、左心房肥大;36例（85.7％）少到中量心包积液、27例（64.3％）左心室射血分数下降.结论 对于原因不明的心功能不全患者,心脏彩色超声检查提示心肌肥厚,但心室大小正常且射血分数下降,心电图提示肢体导联低电压,应怀疑心肌淀粉样变性的可能,必要时进一步行腹部、唇腺或心内膜活检.Objective To characterize the clinical features of patients with cardiac amyloidosis （CA）.Methods Totally 42 patients with CA admitted to Guangdong General Hospital since 2008 were included and retrospectively analyzed in the present study.CA was confirmed by abdomen and endocardium biopsy examination.Clinical manifestations,electrocardiogram and echocardiography were collected for the evaluation.Results Several clinic features are common in CA.In the present study,37 cases （88.1%） presented with chest tightness,dyspnea,20 cases（47.6%） with chest pain,27 cases（64.3%） with right heart failure,27 cases （64.3%） with fatigue,and 30 cases （71.4%） with renal insufficiency and proteinuria.Electrocardiogram （ECG） showed that 32 of the patients （76.2%） were with low voltage in limb leads,29 cases （69%） of them were with poor R wave progression in precordial leads,17 cases （40.5%） with ST-T change,28 cases（66.7%） with pseudo-necrotic Q wave and 36 cases （85.7%） with various kinds of arrhythmia.Echocardiography indicated that all of the subjects （100%） were with different degrees of left ventricular posterior wall or ventricular septal thickness,and left atrial hypertrophy with different degree of myocardial grain appearance or ground-glass opacity.Thirty-six cases （85.7%） were with pericardial effusion,and 27 cases （64.3%） were with abnormal left ventricular eject function.Conclusion For those who were with unexplained clinical cardiac insufficiency,renal insufficiency,myocardial hypertrophy,but normal of ventricular size in echocardiography and low voltage on ECG limb leads,a tissue biopsy from abdomen,labial glands or endocardium should be considered in the diagnosis of CA.

关 键 词：淀粉样变性 心肌 

分 类 号：R542.2[医药卫生—心血管疾病]