Unrecognized wild-type transthyretin amyloidosis may be present in up to 10% to 15% of older adults with heart failure, highlighting the need for early noninvasive diagnosis and emerging therapies.
Older adults with heart failure, specifically focusing on transthyretin amyloid cardiomyopathy (ATTR-CM)
Transthyretin amyloid cardiomyopathy is an under-recognized cause of heart failure in older adults that can now be diagnosed noninvasively and treated with disease-modifying pharmaceutical therapies.
甲状腺素转运蛋白淀粉样变性心肌病(ATTR-CM)是老年人心力衰竭(HF)中一种未被充分认识的病因,源于错误折叠的甲状腺素转运蛋白(TTR)或前白蛋白在心肌中的沉积。超声心动图和心脏磁共振的特征性表现可强烈提示该病,但不能诊断确诊。在无单克隆蛋白证据时,可通过无创核医学影像技术进行诊断。淀粉样纤维的形成,源于遗传性ATTR淀粉样变性(hATTR)中的致不稳定突变,或野生型ATTR淀粉样变性(wtATTR)中与年龄相关的过程。最新研究表明多达10%至15%的老年心衰患者可能存在未被识别的wtATTR。相关表现如腕管综合征和腰椎管狭窄可提高怀疑度,并可能为早期诊断提供途径。此前仅能通过器官移植治疗,现在已有药物治疗可减缓或阻止ATTR-CM进展并改善临床结局。早期识别仍然是实现最佳治疗效果的关键。
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Frederick L. Ruberg
Martha Grogan
Mazen Hanna
Journal of the American College of Cardiology
Boston University
Mayo Clinic
Scripps Research Institute
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Ruberg等人在《Transthyretin amyloid cardiomyopathy (ATTR-CM)》中综述指出,未被识别的野生型甲状腺素转运蛋白淀粉样变可能存在于多达10%至15%的老年心力衰竭患者中,强调了早期无创诊断和新兴疗法的必要性。
www.synapsesocial.com/papers/69ed841f27c2c78c57d1e0cc — DOI: https://doi.org/10.1016/j.jacc.2019.04.003
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