Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

BMC Cardiovascular Disorders

Case Report

Atsushi Okada¹  Teruo Noguchi¹  Chisato Izumi¹  Hideaki Kanzaki¹  Takeshi Kitai¹  Makoto Amaki¹  Kengo Kusano¹  Tomoyuki Fujita²  Naoki Tadokoro²  Takashi Kakuta²  Satsuki Fukushima²  Keiko Ohta-Ogo³  Yoshihiko Ikeda³  Emi Tateishi⁴  Yoshiaki Morita⁴ 

[1] Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 6-1 Kishibe-Shimmachi, 564-8565, Suita, Osaka, Japan;Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan;Department of Pathology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan;Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan;

关键词: Amyloidosis;    Cardiac amyloidosis;    Amyloid cardiomyopathy;    Extraventricular;    Aortic stenosis;    Atrial appendage;    Isolated atrial amyloidosis;    Atrial natriuretic peptide;    Atrial natriuretic factor;    ATTR;    TTR;   

DOI  :  10.1186/s12872-023-03319-3

 received in 2022-09-15, accepted in 2023-05-24,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundRecent studies have reported atrial involvement and coexistence of aortic stenosis in transthyretin (ATTR) cardiac amyloidosis (CA). However, pathological reports of extraventricular ATTR amyloid deposits in atrial structures or heart valves are limited, and the clinical implications of ATTR amyloid deposits outside the ventricles are not fully elucidated.Case presentationWe report 3 cases of extraventricular ATTR amyloid deposits confirmed in surgically resected aortic valves and left atrial structures, all of which were unlikely to have significant ATTR amyloidosis infiltrating the ventricles as determined by multimodality evaluation including 99mtechnetium-pyrophosphate scintigraphy, cardiac magnetic resonance, endomyocardial biopsy and their mid-term clinical course up to 5 years. These findings suggested that these were extraventricular ATTR amyloid deposits localized in the aortic valve and the left atrium.ConclusionsWhile long-term observation is required to fully clarify whether these extraventricular ATTR amyloid deposits are truly localized outside the ventricles or are early stages of ATTR-CA infiltrating the ventricles, our 3 cases with multimodality evaluations and mid-term follow up suggest the existence of extraventricular ATTR amyloid deposits localized in the aortic valve and left atrial structures.

【 授权许可】

CC BY   
© The Author(s) 2023

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