Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy

Background: RNA interference therapeutics reduce transthyretin production; however, their effect on hereditary transthyretin amyloid cardiomyopathy (ATTR-CA) remains unclear. We aimed to investigate alterations in technetium-99 m (99mTc)-pyrophosphate (PYP) single-photon emission computed tomography...

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Main Authors: Yi-Hsin Hung, An-Li Yu, Yi-Chieh Chen, Cheng-Hsuan Tsai, Mao-Yuan Su, 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, Kon-Ping Lin, Wen-Chung Yu, Mei-Fang Cheng, Chi-Chao Chao, Yen-Hung Lin
Format: Article
Language:English
Published: Elsevier 2025-04-01
Series:Journal of the Formosan Medical Association
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Online Access:http://www.sciencedirect.com/science/article/pii/S0929664624004819
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author Yi-Hsin Hung
An-Li Yu
Yi-Chieh Chen
Cheng-Hsuan Tsai
Mao-Yuan Su
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
Kon-Ping Lin
Wen-Chung Yu
Mei-Fang Cheng
Chi-Chao Chao
Yen-Hung Lin
author_facet Yi-Hsin Hung
An-Li Yu
Yi-Chieh Chen
Cheng-Hsuan Tsai
Mao-Yuan Su
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
Kon-Ping Lin
Wen-Chung Yu
Mei-Fang Cheng
Chi-Chao Chao
Yen-Hung Lin
author_sort Yi-Hsin Hung
collection DOAJ
description Background: RNA interference therapeutics reduce transthyretin production; however, their effect on hereditary transthyretin amyloid cardiomyopathy (ATTR-CA) remains unclear. We aimed to investigate alterations in technetium-99 m (99mTc)-pyrophosphate (PYP) single-photon emission computed tomography/computed tomography (SPECT/CT) outcomes in patients receiving patisiran or vutrisiran. Methods: We retrospectively identified individuals with hereditary ATTR-CA who received patisiran or vutrisiran. First and second 99mTc-PYP SPECT/CT data, including visual grading, planar heart to contralateral lung (H/CL) ratio, and volumetric heart to lung (H/L) ratio were assessed. Results: Eight patients with hereditary ATTR-CA were enrolled. Cohort A included four patients who underwent their first 99mTc-PYP SPECT/CT imaging at the initiation of small interfering RNA (siRNA) treatment, while cohort B comprised four patients who had been receiving siRNA treatment before their first 99mTc-PYP SPECT/CT imaging (median duration 1281 days). Overall, there were numerical reductions in planar H/CL ratio (1.7 ± 0.2 to 1.6 ± 0.1, p = 0.050) and a significant improvement in volumetric H/L ratio (4.0 ± 0.9 to 3.5 ± 0.4, p = 0.035). Although without significance, subgroup analysis showed more pronounced changes in cohort A for both planar H/CL ratio and volumetric H/L ratio (−20.1 ± 12.6% and −17.1 ± 11.4%) compared to cohort B (−3.3 ± 11.2% and −4.3 ± 12.7%). Conclusion: Our results demonstrated a significant decrease in volumetric H/L ratio in hereditary ATTR-CA patients receiving RNA interference therapeutics.
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spelling doaj-art-c70dae52c9674ca89d8b4e0a210913732025-08-20T02:10:13ZengElsevierJournal of the Formosan Medical Association0929-66462025-04-01124433333910.1016/j.jfma.2024.10.005Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathyYi-Hsin Hung0An-Li Yu1Yi-Chieh Chen2Cheng-Hsuan Tsai3Mao-Yuan Su4Chia-Tung Shun5Hsueh-Wen Hsueh6Jimmy Jyh-Ming Juang7Ming-Jen Lee8Ping-Huei Tseng9Chia-Hua Hsu10Sung-Tsang Hsieh11Chi-Lun Ko12Kon-Ping Lin13Wen-Chung Yu14Mei-Fang Cheng15Chi-Chao Chao16Yen-Hung Lin17Department of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan; Department of Internal Medicine, Division of Cardiology, National Taiwan University Hospital Hsin-Chu Branch, Hsinchu, TaiwanDepartment of Nuclear Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan; National Taiwan University College of Medicine, Graduate Institute of Clinical Medicine, Taipei, TaiwanDepartment of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan; Department of Medical Imaging and Radiological Technology, Yuanpei University of Medical Technology, Hsinchu, TaiwanDepartment of Forensic Medicine and Pathology, National Taiwan University Hospital, Taipei, TaiwanDepartment of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Internal Medicine, Division of Gastroenterology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Nuclear Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Neurology, Taipei Veterans General Hospital, Taipei, TaiwanDepartment of Internal Medicine, College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Cardiovascular Center, Taipei Veterans General Hospital, Taipei, TaiwanDepartment of Nuclear Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan; Institute of Environmental and Occupational Health Sciences, National Taiwan University, Taipei, Taiwan; Corresponding author. Department of Nuclear Medicine, National Taiwan University Hospital, No. 7, Zhongshan S. Rd., Zhongzheng Dist., Taipei, 100, Taiwan.Department of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan; Corresponding author. Department of Neurology, National Taiwan University Hospital, No. 7, Zhongshan S. Rd., Zhongzheng Dist., Taipei, 100, Taiwan.Department of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanBackground: RNA interference therapeutics reduce transthyretin production; however, their effect on hereditary transthyretin amyloid cardiomyopathy (ATTR-CA) remains unclear. We aimed to investigate alterations in technetium-99 m (99mTc)-pyrophosphate (PYP) single-photon emission computed tomography/computed tomography (SPECT/CT) outcomes in patients receiving patisiran or vutrisiran. Methods: We retrospectively identified individuals with hereditary ATTR-CA who received patisiran or vutrisiran. First and second 99mTc-PYP SPECT/CT data, including visual grading, planar heart to contralateral lung (H/CL) ratio, and volumetric heart to lung (H/L) ratio were assessed. Results: Eight patients with hereditary ATTR-CA were enrolled. Cohort A included four patients who underwent their first 99mTc-PYP SPECT/CT imaging at the initiation of small interfering RNA (siRNA) treatment, while cohort B comprised four patients who had been receiving siRNA treatment before their first 99mTc-PYP SPECT/CT imaging (median duration 1281 days). Overall, there were numerical reductions in planar H/CL ratio (1.7 ± 0.2 to 1.6 ± 0.1, p = 0.050) and a significant improvement in volumetric H/L ratio (4.0 ± 0.9 to 3.5 ± 0.4, p = 0.035). Although without significance, subgroup analysis showed more pronounced changes in cohort A for both planar H/CL ratio and volumetric H/L ratio (−20.1 ± 12.6% and −17.1 ± 11.4%) compared to cohort B (−3.3 ± 11.2% and −4.3 ± 12.7%). Conclusion: Our results demonstrated a significant decrease in volumetric H/L ratio in hereditary ATTR-CA patients receiving RNA interference therapeutics.http://www.sciencedirect.com/science/article/pii/S092966462400481999mTc‐pyrophosphateHereditary transthyretin amyloidosisRNA interference therapeuticsSingle‐photon emission computed tomography/computed tomography
spellingShingle Yi-Hsin Hung
An-Li Yu
Yi-Chieh Chen
Cheng-Hsuan Tsai
Mao-Yuan Su
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
Kon-Ping Lin
Wen-Chung Yu
Mei-Fang Cheng
Chi-Chao Chao
Yen-Hung Lin
Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
Journal of the Formosan Medical Association
99mTc‐pyrophosphate
Hereditary transthyretin amyloidosis
RNA interference therapeutics
Single‐photon emission computed tomography/computed tomography
title Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
title_full Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
title_fullStr Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
title_full_unstemmed Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
title_short Use of technetium-99m-pyrophosphate single-photon emission computed tomography/computed tomography in monitoring therapeutic changes of RNA interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
title_sort use of technetium 99m pyrophosphate single photon emission computed tomography computed tomography in monitoring therapeutic changes of rna interference therapeutics in patients with hereditary transthyretin amyloid cardiomyopathy
topic 99mTc‐pyrophosphate
Hereditary transthyretin amyloidosis
RNA interference therapeutics
Single‐photon emission computed tomography/computed tomography
url http://www.sciencedirect.com/science/article/pii/S0929664624004819
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