Use of Technetium‐99m‐Pyrophosphate Single‐Photon Emission Computed Tomography/Computed Tomography in Monitoring Therapeutic Changes of Eplontersen in Patients With Hereditary Transthyretin Amyloid Cardiomyopathy

An‐Li Yu; Yi‐Chieh Chen; Cheng‐Hsuan Tsai; Yuan‐Kun Aden Wu; Mao‐Yuan Su; Chia‐Hung Chou; Chia‐Tung Shun; Hsueh‐Wen Hsueh; Jimmy Jyh‐Ming Juang; Ming‐Jen Lee; Ping‐Huei Tseng; Chia‐Hua Hsu; Sung‐Tsang Hsieh; Chi‐Lun Ko; Mei‐Fang Cheng; Chi‐Chao Chao; Yen‐Hung Lin

doi:10.1161/JAHA.123.030512

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease (Jan 2024)

Use of Technetium‐99m‐Pyrophosphate Single‐Photon Emission Computed Tomography/Computed Tomography in Monitoring Therapeutic Changes of Eplontersen in Patients With Hereditary Transthyretin Amyloid Cardiomyopathy

An‐Li Yu,
Yi‐Chieh Chen,
Cheng‐Hsuan Tsai,
Yuan‐Kun Aden Wu,
Mao‐Yuan Su,
Chia‐Hung Chou,
Chia‐Tung Shun,
Hsueh‐Wen Hsueh,
Jimmy Jyh‐Ming Juang,
Ming‐Jen Lee,
Ping‐Huei Tseng,
Chia‐Hua Hsu,
Sung‐Tsang Hsieh,
Chi‐Lun Ko,
Mei‐Fang Cheng,
Chi‐Chao Chao,
Yen‐Hung Lin

Affiliations

An‐Li Yu: Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Yi‐Chieh Chen: Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Cheng‐Hsuan Tsai: Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Yuan‐Kun Aden Wu: Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Mao‐Yuan Su: Department of Medical Imaging National Taiwan University Hospital Taipei Taiwan
Chia‐Hung Chou: Department of Obstetrics and Gynecology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Chia‐Tung Shun: Department of Forensic Medicine and Pathology National Taiwan University Hospital Taipei Taiwan
Hsueh‐Wen Hsueh: Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Jimmy Jyh‐Ming Juang: Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Ming‐Jen Lee: Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Ping‐Huei Tseng: Department of Internal Medicine, Division of Gastroenterology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Chia‐Hua Hsu: Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Sung‐Tsang Hsieh: Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Chi‐Lun Ko: Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Mei‐Fang Cheng: Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Chi‐Chao Chao: Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
Yen‐Hung Lin: Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

DOI: https://doi.org/10.1161/JAHA.123.030512
Journal volume & issue: Vol. 13, no. 2

Abstract

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Background Hereditary transthyretin amyloid cardiomyopathy (hATTR‐CM) is a progressive and fatal disease. Recent evidence indicates that bone scintigraphy may serve as a tool to monitor the effectiveness of hATTR‐CM treatment. The objective of this study was to examine how eplontersen therapy influences the semiquantitative uptake of technetium‐99m‐pyrophosphate in individuals diagnosed with hATTR‐CM. Methods and Results We retrospectively analyzed a prospective cohort from the NEURO‐TTRansform trial, including patients with hATTR‐CM receiving eplontersen (45 mg/4 weeks). A control group comprised patients with hATTR‐CM who had not received eplontersen, inotersen, tafamidis, or patisiran. Technetium‐99m‐pyrophosphate single‐photon emission computed tomography/computed tomography was conducted at baseline and during follow‐up. Thirteen patients with hATTR‐CM were enrolled, with 6 receiving eplontersen and 7 serving as the control group. The median follow‐up time was 544 days. The eplontersen group exhibited a significant decrease in volumetric heart and lung ratio (3.774 to 2.979, P=0.028), whereas the control group showed no significant change (4.079 to 3.915, P=0.237). Patients receiving eplontersen demonstrated a significantly greater reduction in volumetric heart and lung ratio compared with the control group (−20.7% versus −3.4%, P=0.007). Conclusions The volumetric heart and lung ratio used to quantify technetium‐99m‐pyrophosphate uptake showed a significant reduction subsequent to eplontersen treatment in individuals diagnosed with hATTR‐CM. These findings suggest the potential efficacy of eplontersen in treating hATTR‐CM and highlight the value of technetium‐99m‐pyrophosphate single‐photon emission computed tomography/computed tomography as a tool for monitoring therapeutic effectiveness.

Published in Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

ISSN: 2047-9980 (Online)
Publisher: Wiley
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the circulatory (Cardiovascular) system
Website: https://www.ahajournals.org/journal/jaha

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