Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
ESC Heart Fail. 2023 Apr;10(2):1412-1417. doi: 10.1002/ehf2.14220. Epub 2022 Nov 6.
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.
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。