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转甲状腺素蛋白淀粉样变性在韩国射血分数保留或轻度降低心力衰竭患者中的患病率及特征。

Transthyretin amyloidosis prevalence and characteristics in Korean patients with heart failure with preserved or mildly reduced ejection fractions.

机构信息

Division of Cardiology, Severance Hospital, Cardiovascular Research Institute, Yonsei University College of Medicine, 50-1, Yonsei-Ro, Seodaemun-gu, Seoul, 03722, Korea.

Department of Nuclear Medicine, Yonsei University College of Medicine, 50-1, Yonsei-Ro, Seodaemun- gu, Seoul, 03722, Korea.

出版信息

Sci Rep. 2024 Oct 1;14(1):22863. doi: 10.1038/s41598-024-74191-0.

Abstract

The diagnosis and awareness of transthyretin amyloidosis cardiomyopathy (ATTR-CM) in heart failure with left ventricular ejection fraction (LVEF) > 40% remains under-recognized. This study aimed to investigate the prevalence and characteristics of ATTR-CM in patients with heart failure with LVEF > 40%. Patients with LVEF > 40% and maximal left ventricular wall thickness (MWT) > 10 mm who underwent bone scintigraphy were retrospectively investigated. Patients with a definite cause of heart failure were excluded. ATTR-CM was diagnosed when grade 2 or 3 myocardial uptake was observed on scintigraphy. Among 97 patients (male, 62.5%; median age, 69 years), 13 (13.4%) were diagnosed with ATTR-CM (wild type, 69.2%; hereditary type, 30.8%). Age or biomarker levels did not differ significantly; however, all patients with ATTR-CM were male. The ATTR-CM group had a significantly higher prevalence of polyneuropathy or carpal tunnel syndrome than the non-ATTR-CM group, accompanied by a longer PR interval, thicker MWT, larger left atrial volume index, and higher E/e'. Accordingly, ATTR was present in a substantial number, particularly among men. Clinicians should suspect ATTR when a male patient exhibits neurologic symptoms, diastolic dysfunction, and a long PR interval.

摘要

转甲状腺素蛋白淀粉样变性心肌病(ATTR-CM)在左心室射血分数(LVEF)>40%的心衰患者中的诊断和认识仍未得到充分重视。本研究旨在探讨 LVEF>40%的心衰患者中 ATTR-CM 的患病率和特征。回顾性分析了 LVEF>40%且最大左心室壁厚度(MWT)>10mm 并接受过骨闪烁显像的患者。排除有明确心衰病因的患者。当闪烁显像观察到 2 级或 3 级心肌摄取时诊断为 ATTR-CM。在 97 例患者中(男性 62.5%;中位年龄 69 岁),13 例(13.4%)诊断为 ATTR-CM(野生型 69.2%;遗传性型 30.8%)。年龄或生物标志物水平无显著差异;然而,所有 ATTR-CM 患者均为男性。ATTR-CM 组的多发性神经病或腕管综合征患病率明显高于非-ATTR-CM 组,伴有更长的 PR 间期、更厚的 MWT、更大的左心房容积指数和更高的 E/e'。因此,大量患者存在 ATTR,特别是男性。当男性患者出现神经症状、舒张功能障碍和较长的 PR 间期时,临床医生应怀疑存在 ATTR。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/876c/11445427/0b47b1c82d4f/41598_2024_74191_Fig1_HTML.jpg

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