摘要
目的:比较轻链型心肌淀粉样变性(AL-CA)和转甲状腺素蛋白心肌淀粉样变性(ATTR-CA)的临床特征。方法:回顾性收集2011年1月至2023年10月在北京大学第三医院住院确诊的AL-CA患者35例(AL-CA组)和ATTR-CA患者21例(ATTR-CA组)。比较两组临床表现、心电图、超声心动图和心脏磁共振成像(CMR)结果的差异。结果:与ATTR-CA组相比,AL-CA组男性比例较低(90.5%vs.54.3%,P=0.005),合并高血压的患者比例较高(9.5%vs.42.9%,P=0.009)。临床表现方面,AL-CA组水肿、蛋白尿、肾功能不全和浆膜腔积液较多见,血红蛋白和血浆白蛋白水平较低,而ATTR-CA组肢体麻木的发生率较高(P均<0.05)。心电图方面,AL-CA组的肢体导联低电压的发生率(57.1%vs.28.6%,P=0.038)高于ATTR-CA组,传导阻滞的发生率(14.3%vs.61.9%,P=0.001)低于ATTR-CA组。AL-CA组从首发症状到确诊时间较ATTR-CA组短[6.0(2.0,15.0)个月vs.35.0(14.0,56.5)个月,P=0.002]。AL-CA组与ATTR-CA组的超声心动图指标室间隔厚度分别为(13.3±2.0)mm vs(.15.7±2.2)mm(P=0.001);左心室质量分别为(198.4±67.8)g vs.(246.6±53.5)g(P=0.009);CMR指标室间隔厚度分别为(16.0±2.1)mm vs.(18.9±3.8)mm(P=0.033);左心室质量分别为(132.9±45.3)g vs.(194.7±50.8)g(P=0.011),AL-CA组左心室壁增厚程度较ATTR-CA组轻,但AL-CA组NYHA心功能分级Ⅲ~Ⅳ级患者的比例较高(48.6%vs.19.0%,P=0.027),N末端B型利钠肽原和心肌肌钙蛋白T水平较高(P均<0.05),1年生存率较低(65.7%vs.100%,P=0.001)。结论:AL-CA和ATTR-CA的临床特征和心电图表现不同。AL-CA左心室壁增厚程度虽然不及ATTR-CA显著,但心功能及预后更差。
Objectives:This study aimed to compare the clinical characteristics between light-chain cardiac amyloidosis(AL-CA)and transthyretin cardiac amyloidosis(ATTR-CA).Methods:A retrospective analysis was conducted in patients diagnosed with AL-CA and ATTR-CA at Peking University Third Hospital from January 2011 to October 2023.There were 35 patients diagnosed with AL-CA(AL-CA group)and 21 patients diagnosed with ATTR-CA(ATTR-CA group).Clinical manifestations,ECG,echocardiography and cardiac magnetic resonance(CMR)results were compared between patients with AL-CA and ATTR-CA.Results:Compared to the AL-CA group,the ATTR-CA group has a higher proportion of males(54.3%vs.90.5%,P=0.005),the proportion of patients with hypertension was lower(42.9%vs.9.5%,P=0.009).In terms of clinical manifestations,edema,proteinuria,renal insufficiency and serous effusion were more common in the AL-CA group,while the incidence of limb numbness was higher in the ATTR-CA group(all P<0.05).There were significant differences in the incidence of limb lead low voltage(57.1%vs.28.6%,P=0.038)and conduction block(14.3%vs.61.9%,P=0.001)between the AL-CA group and ATTR-CA group on ECG.Although the AL-CA group had a shorter time interval from initial symptoms to diagnosis(6.0[2.0,15.0]months vs.35.0[14.0,56.5]months,P=0.002)and less pronounced left ventricular thickening on echocardiography(interventricular septum thickness:[13.3±2.0]mm vs.[15.7±2.2]mm,P=0.001;left ventricular mass:[198.4±67.8]g vs.[246.6±53.5]g,P=0.009)and CMR(interventricular septum thickness:[16.0±2.1]mm vs.[18.9±3.8]mm,P=0.033;left ventricular mass:[132.9±45.3]g vs.[194.7±50.8]g,P=0.011),the proportion of patients with NYHA grades III-IV(48.6%vs.19.0%,P=0.027)was higher,the levels N-terminal B-type natriuretic peptide precursor and cardiac troponin T levels were higher(both P<0.05),and the 1-year survival rate was lower in the AL-CA group than in the ATTR-CA group(65.7%vs.100%,P=0.001).Conclusions:AL-CA and ATTR-CA had differences in clinical and ECG manifestations.While the severity of left ventricular hypertrophy was less pronounced in AL-CA compared to ATTR-CA,cardiac function and prognosis were worse in AL-CA than in ATTR-CA.
作者
陈少敏
林璋
陈宝霞
李璞
冯新恒
CHEN Shaomin;LIN Zhang;CHEN Baoxia;LI Pu;FENG Xinheng(Department of Cardiology,Peking University Third Hospital,NHC Key Laboratory of Cardiovascular Molecular Biology and Regulatory Peptides,Beijing 100191,China;Department of Geriatrics,Fujian Provincial Geriatric Hospital,Fuzhou 350001,China)
出处
《中国循环杂志》
CSCD
北大核心
2024年第7期688-694,共7页
Chinese Circulation Journal
基金
福建省自然科学基金资助项目(2022J01434)
北京大学第三医院临床队列建设项目(BYSYDL 2023009)。
