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左心室射血分数范围内合并肥厚的急性心力衰竭患者中甲状腺转运蛋白淀粉样心肌病的患病率

Prevalence of Transthyretin Amyloid Cardiomyopathy Among Acute Heart Failure Patients with Hypertrophy Across the Left Ventricular Ejection Fraction Spectrum.

作者信息

Velliou Maria, Markos Lampros, Qiuris Stella, Bezati Sofia, Ventoulis Ioannis, Matsiras Dionysis, Bistola Vasiliki, Ikonomidis Ignatios, Polyzogopoulou Effie, Parissis John T

机构信息

University Department of Emergency Medicine, Attikon University Hospital, National and Kapodistrian University of Athens, 12462 Athens, Greece.

Department of Occupational Therapy, University of Western Macedonia, 50200 Ptolemaida, Greece.

出版信息

J Clin Med. 2024 Nov 24;13(23):7103. doi: 10.3390/jcm13237103.

Abstract

Transthyretin amyloid (ATTR) cardiomyopathy mimics left ventricular hypertrophy (LVH) and has been identified as a specific cause of heart failure (HF). The aim of this study was to assess the prevalence of ATTR among patients presenting to the Emergency Department (ED) with acute HF (AHF) and LVH and explore their clinical characteristics and outcomes. Of 127 AHF patients with LVH, 95 completed the diagnostic protocol, which included monoclonal paraprotein testing and technetium-99 m pyrophosphate scintigraphy. Patients were followed for 6 months, and adverse events, including mortality and HF-related hospitalizations, were recorded. ATTR was diagnosed in 8.4% of patients. The mean left ventricular ejection fraction (EF) was 46 ± 7% in ATTR subjects, with 25% classified as HF with reduced EF, 37.5% HF with mildly reduced EF, and 37.5% HF with preserved EF. N-terminal pro b-type natriuretic peptide (NT-proBNP) and high sensitivity troponin T (hs-TnT) were higher in ATTR compared to the non-ATTR group [NT-proBNP: 5863 (6519-12382) pg/mL versus 3586 (1393.5-6322) pg/mL, = 0.007; hs-TnT: 35.9 (47.9-83.8) pg/mL versus 30.0 (19.4-49.5) pg/mL, = 0.0006]. During follow-up, twenty-three patients from the cohort died: six in the ATTR and seventeen in the non-ATTR group. The estimated survival rate was significantly lower in ATTR versus non-ATTR patients (log-rank < 0.0001). In this cohort of AHF patients with LVH presenting to the ED, ATTR cardiomyopathy was detected in 8.4%. Using routinely used cardiac biomarkers and basic echocardiography allows for the raising of suspicion of the disease from the ED setting, potentially facilitating earlier diagnosis in this population.

摘要

转甲状腺素蛋白淀粉样变性(ATTR)心肌病可表现为左心室肥厚(LVH),并已被确认为心力衰竭(HF)的一种特定病因。本研究旨在评估因急性心力衰竭(AHF)和LVH就诊于急诊科(ED)的患者中ATTR的患病率,并探讨其临床特征和预后。在127例合并LVH的AHF患者中,95例完成了诊断方案,其中包括单克隆副蛋白检测和锝-99m焦磷酸盐闪烁扫描。对患者进行了6个月的随访,并记录了不良事件,包括死亡率和与HF相关的住院情况。8.4%的患者被诊断为ATTR。ATTR患者的平均左心室射血分数(EF)为46±7%,其中25%被归类为射血分数降低的HF,37.5%为射血分数轻度降低的HF,37.5%为射血分数保留的HF。与非ATTR组相比,ATTR患者的N末端B型利钠肽原(NT-proBNP)和高敏肌钙蛋白T(hs-TnT)更高[NT-proBNP:5863(6519-12382)pg/mL对3586(1393.5-6322)pg/mL,P=0.007;hs-TnT:35.9(47.9-83.8)pg/mL对30.0(19.4-49.5)pg/mL,P=0.0006]。随访期间,该队列中有23例患者死亡:ATTR组6例,非ATTR组17例。ATTR患者的估计生存率显著低于非ATTR患者(对数秩检验P<0.0001)。在这群因AHF和LVH就诊于ED的患者中,8.4%检测出ATTR心肌病。使用常规的心脏生物标志物和基本超声心动图有助于在ED环境中提高对该病的怀疑,有可能促进该人群的早期诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1992/11642681/54c147b9aea8/jcm-13-07103-g001.jpg

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