一项多中心回顾性队列研究:轻链型和转甲状腺素型心脏淀粉样变性的比较结果和心脏影像学特征。

IF 2.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
Hassan Mahmood, Julius Albert Kato
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引用次数: 0

摘要

心脏淀粉样变性,最常见的表现为免疫球蛋白轻链(AL)和野生型转甲状腺素(atr -wt)亚型,具有独特的病理生理特征和预后。准确区分这些形式对患者管理至关重要。我们对美国南部浸信会卫生系统2015年至2022年间诊断的200例患者(100例AL, 100例atr -wt)进行了一项多中心回顾性队列研究。临床,生化,超声心动图和心脏磁共振成像资料系统回顾。轻链淀粉样变患者比经甲状腺蛋白淀粉样变患者更年轻,表现出更高的血清proBNP,更低的左心室射血分数,以及更大的整体纵向应变损伤。成像模式也不同,AL中主要是心内膜下晚期钆增强,而atr -wt中更常见的是跨壁增强。临床上,AL患者的心力衰竭住院率明显较高,两年生存率较差。总之,轻链型和转甲状腺素型心脏淀粉样变性之间存在显著的人口统计学、影像学和预后差异,强调了多模式评估对指导及时和有针对性的管理策略的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Comparative Outcomes and Cardiac Imaging Features in Light Chain Versus Transthyretin Cardiac Amyloidosis: A Multi-Center Retrospective Cohort Study.

Cardiac amyloidosis, most commonly presenting as immunoglobulin light-chain (AL) and wild-type transthyretin (ATTR-wt) subtypes, has distinct pathophysiological features and prognoses. Accurate differentiation between these forms is critical for patient management. We conducted a multi-center retrospective cohort study of 200 patients (100 AL, 100 ATTR-wt) diagnosed between 2015 and 2022 within the Baptist Health System in the Southern United States. Clinical, biochemical, echocardiographic, and cardiac magnetic resonance imaging data were systematically reviewed. Patients with light-chain amyloidosis were younger, exhibited higher serum proBNP, lower left ventricular ejection fraction, and greater impairment in global longitudinal strain compared with those with transthyretin amyloidosis. Imaging patterns also differed, with subendocardial late gadolinium enhancement predominating in AL and transmural enhancement more frequent in ATTR-wt. Clinically, AL patients experienced substantially higher heart failure hospitalization rates and worse two-year survival. In conclusion, significant demographic, imaging, and outcome differences exist between light-chain and transthyretin cardiac amyloidosis, highlighting the importance of multimodal assessment to guide timely and targeted management strategies.

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来源期刊
American Journal of Cardiology
American Journal of Cardiology 医学-心血管系统
CiteScore
4.00
自引率
3.60%
发文量
698
审稿时长
33 days
期刊介绍: Published 24 times a year, The American Journal of Cardiology® is an independent journal designed for cardiovascular disease specialists and internists with a subspecialty in cardiology throughout the world. AJC is an independent, scientific, peer-reviewed journal of original articles that focus on the practical, clinical approach to the diagnosis and treatment of cardiovascular disease. AJC has one of the fastest acceptance to publication times in Cardiology. Features report on systemic hypertension, methodology, drugs, pacing, arrhythmia, preventive cardiology, congestive heart failure, valvular heart disease, congenital heart disease, and cardiomyopathy. Also included are editorials, readers'' comments, and symposia.
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