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-Reference-Cited by-同舟云学术

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

Published:2024-01-16 Issue:2 Volume:13 Page:
ISSN:2047-9980
Container-title:Journal of the American Heart Association
language:en
Short-container-title:JAHA

Author:

Yu An‐Li¹²^ORCID,Chen Yi‐Chieh³,Tsai Cheng‐Hsuan¹⁴^ORCID,Wu Yuan‐Kun Aden¹,Su Mao‐Yuan⁵⁶^ORCID,Chou Chia‐Hung⁷,Shun Chia‐Tung⁸^ORCID,Hsueh Hsueh‐Wen⁹^ORCID,Juang Jimmy Jyh‐Ming¹^ORCID,Lee Ming‐Jen⁹,Tseng Ping‐Huei¹⁰,Hsu Chia‐Hua⁹^ORCID,Hsieh Sung‐Tsang⁹^ORCID,Ko Chi‐Lun³,Cheng Mei‐Fang³¹¹^ORCID,Chao Chi‐Chao⁹^ORCID,Lin Yen‐Hung¹^ORCID

Affiliation:

1. Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

2. Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital Hsin‐Chu Branch Hsinchu Taiwan

3. Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

4. National Taiwan University College of Medicine Graduate Institute of Clinical Medicine Taipei Taiwan

5. Department of Medical Imaging National Taiwan University Hospital Taipei Taiwan

6. Department of Medical Imaging and Radiological Technology Yuanpei University of Medical Technology Hsinchu Taiwan

7. Department of Obstetrics and Gynecology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

8. Department of Forensic Medicine and Pathology National Taiwan University Hospital Taipei Taiwan

9. Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

10. Department of Internal Medicine, Division of Gastroenterology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

11. Institute of Environmental and Occupational Health Sciences National Taiwan University Taipei Taiwan

Abstract

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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Cardiology and Cardiovascular Medicine

Reference46 articles.

1. THAOS – The Transthyretin Amyloidosis Outcomes Survey: initial report on clinical manifestations in patients with hereditary and wild-type transthyretin amyloidosis

2. Hereditary transthyretin amyloidosis: a model of medical progress for a fatal disease

3. Amyloidosis as a Systemic Disease in Context

4. Transthyretin (ATTR) amyloidosis: clinical spectrum, molecular pathogenesis and disease-modifying treatments

5. Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis