Abstract

Tafamidis meglumine, a transthyretin (TTR) stabilizer, is effective in delaying the progression of neuropathy in TTR amyloidosis with Val30Met mutations. However, its efficacy in TTR amyloid cardiomyopathy is not fully elucidated. Herein, we report a 73‐year‐old Japanese man with a diagnosis of TTR amyloid cardiomyopathy with Val30Met mutation treated with tafamidis. To evaluate treatment response, cardiac magnetic resonance imaging was performed before and after 12 months of tafamidis treatment. Native T1, extracellular volume, and left ventricular mass showed no obvious worsening, and findings of other diagnostic studies also supported the efficacy of tafamidis to delay the progression of amyloid cardiomyopathy. Our case suggests that serial native T1 and extracellular volume may be novel non‐invasive imaging methods to monitor the treatment response to TTR stabilizers in cardiac amyloidosis and also that tafamidis may be effective in suppressing cardiac progression in TTR amyloid cardiomyopathy with Val30Met mutation.

Details

Title
Monitoring treatment response to tafamidis by serial native T1 and extracellular volume in transthyretin amyloid cardiomyopathy
Author
Shintani, Yasuhiro 1 ; Okada, Atsushi 1 ; Morita, Yoshiaki 2 ; Hamatani, Yasuhiro 1 ; Amano, Masashi 1 ; Takahama, Hiroyuki 1 ; Amaki, Makoto 1 ; Hasegawa, Takuya 1 ; Keiko Ohta‐Ogo 3 ; Kanzaki, Hideaki 1 ; Hatsue Ishibashi‐Ueda 3 ; Yasuda, Satoshi 1 ; Shimazaki, Chihiro 4 ; Yoshinaga, Tsuneaki 5 ; Yazaki, Masahide 6 ; Sekijima, Yoshiki 6 ; Izumi, Chisato 1 

 Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan 
 Department of Radiology, National Cerebral and Cardiovascular Center, Osaka, Japan 
 Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan 
 Department of Hematology, Japan Community Health care Organization Kyoto Kuramaguchi Medical Center, Kyoto, Japan 
 Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan 
 Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan; Department of Biological Sciences for Intractable Neurological Diseases, Institute for Biomedical Sciences, Shinshu University, Matsumoto, Japan 
Pages
232-236
Section
Case Reports
Publication year
2019
Publication date
Feb 2019
Publisher
Oxford University Press
e-ISSN
20555822
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
2290245134
Copyright
© 2019. This work is published under http://creativecommons.org/licenses/by-nc/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.