Comparison of Clinical Characteristics Between Light-chain and Transthyretin Cardiac Amyloidosis
10.3969/j.issn.1000-3614.2024.07.006
- VernacularTitle:轻链型和转甲状腺素蛋白心肌淀粉样变性临床特征的比较
- Author:
Shaomin CHEN
1
;
Zhang LIN
;
Baoxia CHEN
;
Pu LI
;
Xinheng FENG
Author Information
1. 北京大学第三医院 心内科 国家卫生健康委心血管分子生物学与调节肽重点实验室,北京 100191
- Keywords:
infiltrative cardiomyopathy;
light chain cardiac amyloidosis;
transthyretin cardiac amyloidosis
- From:
Chinese Circulation Journal
2024;39(7):688-694
- CountryChina
- Language:Chinese
-
Abstract:
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.
