淀粉样变心肌病的诊断思路  

Clinical consideration of diagnosis in cardiac amyloidosis

作　　者：戴红芬[1] 廖玉华[1] 袁璟[1] 付卿卿[1] 曾淘[1] 文建平[1] 

机构地区：[1]华中科技大学协和医院心内科华中科技大学同济医学院心血管病研究所,武汉430022

出　　处：《临床心血管病杂志》2008年第1期7-9,共3页Journal of Clinical Cardiology

摘　　要：目的:通过对淀粉样变心肌病的临床分析以探讨该病早期诊断的方法。方法:回顾性分析8例原发性淀粉样变心肌病患者的临床特征、心电图及超声心动图表现、确诊方法和预后。结果:临床表现为心力衰竭患者7例,其中右心力衰竭4例,全心力衰竭3例;既往高血压而表现为低血压5例;舌大5例。心电图示:肢体导联低电压6例,其中5例并发前胸导联R波进展不良和ST-T改变。超声心动图示:所有患者均表现有左心室大小正常,左心房明显增大,室间隔和左室壁均明显增厚伴室壁运动弥漫性减弱。8例患者经心内膜下心肌或舌肌取材,通过苏木精-伊红和刚果红染色,均为阳性。平均随访(23.6±11.3)个月,8例患者均死亡。结论:淀粉样变性是一种系统性疾病,并发心肌病变患者有以下临床特征:①心室腔不大伴进行性难治性心力衰竭,②左心室肥厚伴心电图低电压,③既往有高血压伴进行性低血压及类似陈旧性心肌梗死心电图图形,④左室壁均匀肥厚伴室壁活动弥漫性减低,⑤舌体宽大肥厚;通过心内膜心肌或心外组织活检和组织化学染色可以确诊。Objective：To explore the early diagnosis of cardiac amyloidosis by analyzing its clinical features. Method：Eight patients with cardiac involvement associated with primary amyloidosis were studied in this study. The data of clinical characteristics, electrocardiogram （ECG） and echocardiography （UCG） were analyzed. Biopsy specimens of subendocardiac muscle and lingual muscle were observed under light microscope. Prognosis was evaluated by follow-up for several months. Result： The first symptoms of the patients were quite different but most presented as dyspnea. Heart failure was observed in 7 patients （4 cases with right heart failure and 3 cases with whole heart failure）. What＇s more, 5 cases had hypotension but hypertension previously, and 5 had macroglossia. ECG showed that 6 patients were low voltage in the limb leads, combining with poor R wave progression in the precordial leads and ST-T changes in 5 patients. UCG manifested that all of the patients were possessed of normal left ventricle, enlarged left atrium, increased thickness of the interventricular septums and left ventricular walls as well as diffuse weakness of the wall motion. Biopsy of subendocardiac muscle in 2 patients and lingual muscle in another six were collected and stained with HE. Typical deposition of amyloid were found in each one of them. Followed up for （23.6±11.3） months, all of the patients were died. Conclusion：Amyloidosis is a systemic disease whose cardiac involvement will manifest; （1） progressing refractory heart failure with normal ventricular chamber, （2） low-voltage ORS complexes with left ventricular hypertrophy, （3）progressing hypotension but hypertension previously or pseudoinfarction patterns, （4） thickened left ventricular wall with diffusely weakened wall motion, （5） tongue widen and thicken. Biopsy of subendocardiac muscle or extracardiac tissue, such as lingual muscle, remains the definitive diagnostic method for cardiac amyloidosis.

关 键 词：心肌疾病 淀粉样变 

分 类 号：R597.3[医药卫生—内科学]