混合型心肌淀粉样变一例及文献回顾  

Mixed Cardiac Amyloidosis:a Case Report and Literature Review

作　　者：邵兴慧[1] 姚晶 SHAO Xinghui;YAO Jing(Department of Cardiology,National Center for Cardiovascular Diseases and Fuwai Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100037,China;Department of Cardiology,Beijing Anzhen Hospital,Capital Medical University,Beijing 100029,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,阜外医院心内科,北京100037 [2]首都医科大学附属北京安贞医院心内科,北京100013

出　　处：《中国循环杂志》2025年第4期388-391,共4页Chinese Circulation Journal

摘　　要：本文报道了一例罕见的混合型心肌淀粉样变病例。患者66岁女性,因反复晕厥就诊,初诊为梗阻性肥厚型心肌病。随着病情进展,出现多系统受累表现。最终通过心内膜心肌活检确诊为系统性轻链(AL)-λ型和转甲状腺素蛋白型(ATTR)的混合型心肌淀粉样变,以AL-λ型为主。文章回顾了心肌淀粉样变的临床表现、诊断方法和治疗原则,并讨论了混合型淀粉样变的罕见性及其诊断挑战。本例提示临床医师在面对进行性心力衰竭患者时,应保持高度警惕,考虑淀粉样变性可能,并强调了心内膜心肌活检在诊断中的重要性。We report a rare case of mixed cardiac amyloidosis.The patient was a 66-year-old woman who suffered from recurrent syncope and hypertrophic cardiomyopathy was diagnosed.As the disease progresses,multiple systems are involved.Finally,AL-λand transthyretin amylodosis(ATTR)mixed cardiac amyloidosis was confirmed by endocardial myocardial biopsy,mainly AL-λtype.This article reviews the clinical manifestations,diagnostic methods,and therapeutic modalities of cardiac amyloidosis,and discusses the rarity of mixed amyloidosis and its diagnostic challenges.This case suggests that clinicians should remain vigilant in considering the possibility of amyloidosis when dealing with patients with progressive heart failure and cardiac hypertrophy,and highlights the importance of endocardial myocardial biopsy in the diagnosis of cardiac amyloidosis in suspected patients.

关 键 词：心肌淀粉样变 系统性轻链型淀粉样变 转甲状腺素蛋白型淀粉样变 心内膜心肌活检 

分 类 号：R54[医药卫生—心血管疾病]