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无心室肥厚的野生型转甲状腺素蛋白心脏淀粉样变性的多模态评估。

Multimodality assessments of wild-type transthyretin cardiac amyloidosis with no ventricular hypertrophy.

机构信息

Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

出版信息

ESC Heart Fail. 2023 Apr;10(2):1412-1417. doi: 10.1002/ehf2.14220. Epub 2022 Nov 6.

Abstract

While patients with transthyretin cardiac amyloidosis (ATTR-CA) typically present with concentric or asymmetric hypertrophy, a small percentage of ATTR-CA is known to present with 'atypical' cardiac morphologies such as eccentric hypertrophy or even no hypertrophy. However, detailed report of multimodality assessments of ATTR-CA with no ventricular hypertrophy is lacking. Herein, we report detailed multimodality assessments of an 81-year-old Japanese woman with heart failure and history of carpal tunnel syndrome and lumbar canal stenosis, presenting no ventricular hypertrophy and negative technetium-pyrophosphate scintigraphy, who was eventually diagnosed as having wild-type ATTR-CA. Our case highlights the role of multimodality assessments for early diagnosis of ATTR-CA in patients with atypical cardiac morphologies and also emphasizes the limitations of bone scintigraphy and the importance of considering ATTR-CA in patients with non-cardiac manifestations of ATTR amyloidosis.

摘要

尽管转甲状腺素蛋白心脏淀粉样变(ATTR-CA)患者通常表现为向心性或不对称性肥厚,但有一小部分 ATTR-CA 已知表现为“非典型”的心脏形态,如偏心性肥厚,甚至无肥厚。然而,缺乏对无心室肥厚的 ATTR-CA 的详细多模式评估报告。在此,我们报告了一位 81 岁日本女性的详细多模式评估,该女性患有心力衰竭和腕管综合征及腰椎管狭窄病史,无心室肥厚且焦磷酸锝[99mTc]扫描阴性,最终被诊断为野生型 ATTR-CA。我们的病例强调了多模式评估在诊断具有非典型心脏形态的 ATTR-CA 患者中的作用,也强调了骨闪烁扫描的局限性和在具有非心脏 ATTR 淀粉样变性表现的患者中考虑 ATTR-CA 的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/449b/10053176/3261de5bffc3/EHF2-10-1412-g002.jpg

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