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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 method of early diagnosis of amyloid cardiomyopathy through clinical analysis. Methods: The clinical features, electrocardiogram (ECG), echocardiographic findings, diagnosis and prognosis of 8 patients with primary amyloid cardiomyopathy were analyzed retrospectively. Results: The clinical manifestations of 7 cases of heart failure patients, including 4 cases of right heart failure, heart failure in 3 cases; previous hypertension showed hypotension in 5 cases; tongue in 5 cases. ECG: limb lead low voltage in 6 cases, of which 5 cases of complicated precordial lead R wave bad progress and ST-T changes. Echocardiography: All patients showed normal left ventricular size, left atrial significantly increased, ventricular septum and left ventricular wall were significantly thickening with diffuse wall motion decreased. Eight patients were drawn from subendocardial myocardium or lingual muscle and were positive for hematoxylin-eosin and congo red staining. The average follow-up (23.6 ± 11.3) months, 8 patients died. CONCLUSIONS: Amyloidosis is a systemic disease that has the following clinical features in patients with myocardial lesions: ventricular chamber insufficiency associated with refractory heart failure, left ventricular hypertrophy with ECG low voltage, previous high blood pressure associated with Hypotension and similar old myocardial infarction ECG patterns, ④ homogeneous hypertrophy of the left ventricular wall with diffuse reduction of ventricular activity, ⑤ large tongue hypertrophy; by endomyocardial or extracardiac biopsy and histochemical staining can be confirmed.