心肌淀粉样变在心肌肥厚患者中的检出率及临床特点与预后分析  

Proportion,Clinical Features and Prognosis of Cardiac Amyloidosis in Patients With Myocardial Hypertrophy

作　　者：秦莹 孙筱璐[1] 蒋文[1] 王东[1] 毛懿[2] 张沫 许连军[1] 王丽梅[1] 李剑 宋雷[1] 康连鸣[1] QIN Ying;SUN Xiaolu;JIANG Wen;WANG Dong;MAO Yi;ZHANG Mo;XU Lianjun;WANG Limei;LI Jian;SONG Lei;KANG Lianming(Department of Cardiomyopathy,National Center for Cardiovascular Diseases and Fuwai Hospital,CAMS and PUMC,Beijing(100037),China)

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外医院心肌病病区,北京市100037 [2]中国医学科学院北京协和医学院国家心血管病中心阜外医院心力衰竭中心,北京市100037 [3]中国医学科学院北京协和医学院北京协和医院血液科

出　　处：《中国循环杂志》2022年第6期602-608,共7页Chinese Circulation Journal

基　　金：国家自然科学基金(81870286);中国医学科学院临床与转化医学研究基金(2020-12M-C&T-A-006)。

摘　　要：目的:探讨心肌肥厚患者中心肌淀粉样变(CA)的检出率,并分析其临床特点与预后。方法:回顾分析2020年4月至2021年1月在中国医学科学院阜外医院心肌病病区住院的心肌肥厚患者中,经组织病理确诊CA的患者比例、临床表现、辅助检查及预后。结果:373例心肌肥厚患者中,21例(5.6%)经组织病理活检证实为CA。其中,免疫球蛋白轻链型心肌淀粉样变(AL-CA)19例(5.1%),转甲状腺素蛋白型心肌淀粉样变(ATTR-CA)2例(0.5%)。患者平均年龄(58.2±9.2)岁,男性17例(81.0%),就诊症状主要为胸闷、气短19例(90.5%),晕厥5例(23.8%),浮肿4例(19.1%);中位病程7(6,13)个月。AL-CA根据梅奥2012分期,Ⅲ期7例(36.8%),Ⅳ期11例(57.9%)。2例ATTR-CA分期均为Ⅱ期。心电图以肢体导联QRS低电压(16例,76.2%)、胸前导联R波递增不良(15例,71.4%)为主;超声心动图双心房增大(13例,61.9%),左心室射血分数(56.5±9.3)%,室间隔厚度(16.9±3.4)mm,舒张功能减低21例(100%),心包积液16例(76.2%);心脏磁共振成像(CMR)示心内膜弥漫延迟强化21例(100%)。中位随访3.1(1.8,6.4)个月,7例(33.3%)死亡,均为AL-CA患者。中位总生存时间7个月。经Cox回归分析,校正年龄、性别后,左心房前后径仍是AL-CA患者死亡的独立预测因素(HR=1.20,95%CI:1.01~1.44,P=0.042)。结论:CA在心肌肥厚患者中并不罕见。心电图呈肢导低电压、超声心动图示双心房增大及心包积液、CMR示心内膜弥漫延迟强化,需怀疑CA。左心房前后径是AL-CA患者死亡的独立预测因素。Objectives:To investigate the proportion,clinical features and prognosis of cardiac amyloidosis(CA)in patients with myocardial hypertrophy.Methods:In this retrospective study,we explored the proportion of CA confirmed by histopathology in patients with myocardial hypertrophy,who were hospitalized in our center from April 2020 to January 2021,and analyzed their clinical manifestations,imaging examination results and prognosis.Results:CA was confirmed in 21(5.6%)out of 373 patients with myocardial hypertrophy by histopathological biopsy.There were 19 cases(5.1%)of light chain CA(AL-CA),and 2 cases(0.5%)of transthyretin CA(ATTR-CA).The average age was(58.2±9.2)years,there were 17 male patients(81.0%).The main symptoms were chest tightness(90.5%),syncope(23.8%),and edema(19.1%).The mean duration of the disease was 7(6-13)months.The average blood pressure was 103/68 mmHg.In AL-CA,according to the Mayo 2012 staging standard,stageⅢpatients accounted for 36.8%,and stageⅣpatients accounted for 57.9%.Both cases of ATTR-CA were stage 2 patients.The main manifestations of ECG were low QRS voltage of limb lead(76.2%)and poor increment of chest lead R wave(71.4%).Echocardiography examination revealed right and left atrial enlargement(61.9%),mean left ventricular ejection fraction was(56.5±9.3)%,mean interventricular septum thickness was(16.9±3.4)mm,ratio of diastolic dysfunction was 100%,proportion of pericardial effusion was 76.2%;cardiac MRI showed diffuse subendocardial delayed-enhanced gadolinium imaging in all CA patients(100%).The mean follow-up time was 3.1(1.8-6.4)months,and 7 AL-CA cases(33.3%)died during follow up,and the median overall survival time was 7.0 months in these 7 patients.After adjusting for age and sex,Cox regression analysis showed that left atrial anteroposterior diameter remained as an independent predictor of death in AL-CA patients(HR=1.20,95%CI:1.01-1.44,P=0.042).Conclusions:CA is not uncommon in patients with myocardial hypertrophy,and the mortality rate is high.The main electrocardiogram

关 键 词：淀粉样变 心肌病 肥厚 临床特点 预后 

分 类 号：R54[医药卫生—心血管疾病]