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转甲状腺素蛋白心脏淀粉样变:心力衰竭的广泛表型谱及其诊断意义

Transthyretin cardiac amyloid: Broad heart failure phenotypic spectrum and implications for diagnosis.

作者信息

Alonso Mileydis, Neicheril Radhika K, Manla Yosef, McDonald Malcolm L, Sanchez Alejandro, Lafave Gabrielle, Seijo De Armas Yelenis, Camargo Antonio Lewis, Uppal Dipan, Wolinsky David, Thakkar-Rivera Nina, Velez Mauricio, Baran David A, Estep Jerry D, Snipelisky David

机构信息

Department of Cardiovascular Disease, Heart, Vascular, and Thoracic Institute, Cleveland Clinic Florida, Weston, Florida, USA.

Department of Medicine, Internal Medicine, Cleveland Clinic Florida, Weston, Florida, USA.

出版信息

ESC Heart Fail. 2024 Dec;11(6):3649-3655. doi: 10.1002/ehf2.15035. Epub 2024 Aug 24.

DOI:10.1002/ehf2.15035
PMID:39180423
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11631306/
Abstract

AIMS

Transthyretin cardiac amyloidosis (ATTR-CA) is most often associated with heart failure with preserved ejection fraction (HFpEF). However, patients may present with impaired systolic function at the time of diagnosis, which has not been widely investigated. We sought to explore the prevalence of various heart failure (HF) phenotypes and their associated clinical characteristics at the time of ATTR-CA diagnosis.

METHODS

We performed a single-centre retrospective cohort study of consecutive patients with ATTR-CA evaluated between February 2016 and December 2022. Data on patient demographics, comorbidities, imaging and laboratory findings were compared across HF phenotypes (age: 78.1 ± 8.6 years, with 91.1% male). A total of 21.6% (n = 46) presented with heart failure with reduced ejection fraction (HFrEF), 17.8% (n = 38) with heart failure with mildly reduced ejection fraction (HFmrEF) and 60.6% (n = 129) with HFpEF at the time of diagnosis with ATTR-CA. Those presenting with HFrEF or HFmrEF were more likely to be African American and had significantly worse New York Heart Association (NYHA) functional class, higher N-terminal pro-brain natriuretic peptide (NT-proBNP) and higher serum creatinine levels as compared with those with HFpEF.

CONCLUSIONS

Although ATTR-CA is traditionally thought to be seen primarily among patients with HFpEF, our data suggest that ATTR-CA has a higher prevalence among patients with HFrEF, which underscores the importance of heightened clinical suspicion regardless of ejection fraction when considering ATTR-CA. Furthermore, although comorbidities are similar, patients with HFmrEF and HFrEF had a worse symptom burden.

摘要

目的

转甲状腺素蛋白心脏淀粉样变性(ATTR-CA)最常与射血分数保留的心力衰竭(HFpEF)相关。然而,患者在诊断时可能出现收缩功能受损,这一点尚未得到广泛研究。我们试图探讨ATTR-CA诊断时各种心力衰竭(HF)表型的患病率及其相关临床特征。

方法

我们对2016年2月至2022年12月期间连续评估的ATTR-CA患者进行了单中心回顾性队列研究。比较了不同HF表型患者的人口统计学、合并症、影像学和实验室检查结果(年龄:78.1±8.6岁,男性占91.1%)。在ATTR-CA诊断时,共有21.6%(n = 46)的患者表现为射血分数降低的心力衰竭(HFrEF),17.8%(n = 38)的患者表现为射血分数轻度降低的心力衰竭(HFmrEF),60.6%(n = 129)的患者表现为HFpEF。与HFpEF患者相比,表现为HFrEF或HFmrEF的患者更可能是非裔美国人,纽约心脏协会(NYHA)功能分级明显更差,N末端脑钠肽前体(NT-proBNP)更高,血清肌酐水平更高。

结论

尽管传统上认为ATTR-CA主要见于HFpEF患者,但我们的数据表明,ATTR-CA在HFrEF患者中的患病率更高,这凸显了在考虑ATTR-CA时,无论射血分数如何,提高临床怀疑的重要性。此外,尽管合并症相似,但HFmrEF和HFrEF患者的症状负担更重。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c187/11631306/5402fcb97811/EHF2-11-3649-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c187/11631306/5402fcb97811/EHF2-11-3649-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c187/11631306/5402fcb97811/EHF2-11-3649-g001.jpg

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