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