淀粉样变致限制型心肌病的临床特征研究

Clinical characteristics of restrictive cardiomyopathy secondary to amyloidosis

目的:探讨继发于心肌淀粉样变(CA)的成人限制型心肌病(RCM)的临床特征。方法:分析2013~2019年确诊为CA致RCM患者的基本特征、临床症状、心电图及心脏超声表现等。结果:连续入选RCM患者22例,平均年龄(55.36±12.51)岁,其中男性13例(59.09%)。患者纽约心功能分级(NYHA)Ⅲ级和Ⅳ级者占81.82%。12例(54.54%)以胸闷气喘为主,5例(22.72%)以下肢水肿为主,其余有腹胀、晕厥等。68.18%表现为肢体导联低电压,90.91%的胸导联R波递增不良,59.09%的患者出现假性梗死Q波。心脏超声显示,22例患者LVDd(45.62±8.39)mm, LVEF(52.72±12.03)%,IVS13(10,14)mm, 17例(77.28%)LVEF>40%,11例(50.00%)患者出现中重度三尖瓣关闭不全。19例患者Pro-BNP 2 933(2 437,5 955)ng/L。结论:继发于CA的RCM患者,多表现为射血分数保留的心衰,具有独特的心电学和超声特征,可为临床诊断提供一定的线索与依据。

Objective:To investigate the clinical characteristics of restrictive cardiomyopathy(RCM)secondary to amyloidosis in adults.Methods:The baseline characteristics,clinical symptoms,parameters of ECG and echocardiography of patients with RCM secondary to cardiac amyloidosis were collected from 2013 to 2019 and retrospectively analyzed.Results:In total,22 consecutive RCM cases were included.Thirteen of them were males(59.09%),with an average age of(55.36±12.51)years.Classification of the cardiac function by NYHA showed that 81.82%patients were in gradeⅢandⅣ.The main clinical symptoms were associated with chest tightness and shortness of breath(n=12 cases,54.54%),edema at lower limbs(n=5 cases,22.72%),abdominal distension and syncope.By electrocardiographic features,68.18%showed low voltage of limb lead,90.91%of the thoracic lead had poor R-wave increase,59.09%had pseudo infarct Q wave.Echocardiographic findings:LVDD(45.62±8.39)mm,LVEF(52.72±12.03)%,IVS 13(10,14)mm,LVEF>40%in 17 cases(77.28%),and moderate to severe tricuspid regurgitation in 11 cases(50%).Pro-BNP level was 2933(2437,5955)ng/L in 19 cases.Conclusion:Patients with RCM secondary to myocardial amyloidosis are usually characterized by heart failure with preserved ejection fraction as well as unique electrocardiographic and ultrasonic pictures,which can provide certain clues and basis for clinical diagnosis of this condition.