心脏淀粉样变患者的心电图和心脏超声特点  

Electrocardiographic and echocardiographic features of patients with primary cardiac amyloidosis

作　　者：程中伟[1] 田庄[1] 康琳[1] 陈太波[1] 方理刚[1] 程康安[1] 曾勇[1] 方全[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院心内科,100730

出　　处：《中华心血管病杂志》2010年第7期606-609,共4页Chinese Journal of Cardiology

摘　　要：目的 总结心内膜心肌活检（EMB）证实原发型心脏淀粉样变（CA）患者的心电图和心脏超声特点,为临床医师能够早期识别和诊断CA提供帮助.方法 自2006年9月至2009年10月收治临床怀疑CA患者共20例（其中男性7例）,平均年龄（50±12）岁,进行EMB检查.11例（55%）患者诊断为CA,血清和（或）尿检查示游离单克隆轻链（λ）明显升高,确诊为原发型CA.分析该11例心电图和心脏超声的特点.结果 心电图分析发现,11例患者的6个肢导联电压均较低,均值为0.33～0.51 mV,其中肢导联低电压和假性梗死波形发生率均为45%.心脏超声检查结果分析发现,11例患者室壁呈向心性增厚和左心室腔容积正常,绝大多数患者可见左心房扩大（10例,91%）、心肌内可见颗粒样强回声（9例,82%）、中至大量心包积液（7例,64%）以及左心室收缩功能受损（8例,73%）.结论 对于临床原因不明的心力衰竭,心脏超声示向心性肥厚且左心室腔容积不大,伴心肌内颗粒样强回声或心包积液,而心电图示肢导联低电压或假性梗死波形者,高度疑似原发型CA的可能性,应进一步行EMB和血清（尿）生化检查,以便早期明确诊断和及时治疗.Objective To summarize the electrocardiography and echocardiography features of patients with cardiac amyloidosis （CA） diagnosed by endo-myocardial biopsy （EMB）. Methods A total of 20 consecutive patients [7 men, mean age （50 ± 12 ）years] referred for EMB because of clinical suspicion of CA from September 2006 to October 2009 were included in the study. Primary CA was diagnosed in 11 out of 20 patients （55% ） by EMB and biomarkers examination. The electrocardiography and echocardiography features were analyzed. Results The voltage of all the limb leads were low in the 11 CA patients [mean values of （0. 33 -0. 51） mV], the incidence of low voltage and pseudo-infarction patterns were 45% and 45% , respectively. Concentric hypertrophy and normal left ventricular diameters were evidenced in all CA patients on echocardiography, left atrial enlargement （n = 10, 91% ） , granular/sparking appearance of the myocardium （n = 9, 82% ） and moderate to large pericardial effusion （n = 7, 64% ） as well as left ventricular systolic dysfunction （ n = 8, 73% ） were often presented in CA patients. Conclusions The diagnosis of primary CA should be considered in patients with unknown origin of heart failure, concentric hypertrophy and normal left ventricular diameters with granular/sparking appearance of the myocardium or pericardial effusion presented on echocardiography and low voltage of limb leads or pseudo-infarction pattern presented on electrocardiography. EMB and serum （urine） biomarkers examinations should be then performed to confirm or exclude the diagnosis of CA.

关 键 词：淀粉样变性 心肌 心电描记术 超声心动描记术 

分 类 号：R541[医药卫生—心血管疾病]