{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T03:45:01Z","timestamp":1793159101676,"version":"4.5.0"},"reference-count":46,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2024,1,16]]},"abstract":"<jats:sec sec-type=\"background\" xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Hereditary transthyretin amyloid cardiomyopathy (hATTR\u2010CM) is a progressive and fatal disease. Recent evidence indicates that bone scintigraphy may serve as a tool to monitor the effectiveness of hATTR\u2010CM treatment. The objective of this study was to examine how eplontersen therapy influences the semiquantitative uptake of technetium\u201099m\u2010pyrophosphate in individuals diagnosed with hATTR\u2010CM.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              We retrospectively analyzed a prospective cohort from the NEURO\u2010TTRansform trial, including patients with hATTR\u2010CM receiving eplontersen (45\u2009mg\/4\u2009weeks). A control group comprised patients with hATTR\u2010CM who had not received eplontersen, inotersen, tafamidis, or patisiran. Technetium\u201099m\u2010pyrophosphate single\u2010photon emission computed tomography\/computed tomography was conducted at baseline and during follow\u2010up. Thirteen patients with hATTR\u2010CM were enrolled, with 6 receiving eplontersen and 7 serving as the control group. The median follow\u2010up time was 544\u2009days. The eplontersen group exhibited a significant decrease in volumetric heart and lung ratio (3.774 to 2.979,\n              <jats:italic>P<\/jats:italic>\n              =0.028), whereas the control group showed no significant change (4.079 to 3.915,\n              <jats:italic>P<\/jats:italic>\n              =0.237). Patients receiving eplontersen demonstrated a significantly greater reduction in volumetric heart and lung ratio compared with the control group (\u221220.7% versus \u22123.4%,\n              <jats:italic>P<\/jats:italic>\n              =0.007).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">The volumetric heart and lung ratio used to quantify technetium\u201099m\u2010pyrophosphate uptake showed a significant reduction subsequent to eplontersen treatment in individuals diagnosed with hATTR\u2010CM. These findings suggest the potential efficacy of eplontersen in treating hATTR\u2010CM and highlight the value of technetium\u201099m\u2010pyrophosphate single\u2010photon emission computed tomography\/computed tomography as a tool for monitoring therapeutic effectiveness.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.123.030512","type":"journal-article","created":{"date-parts":[[2024,1,12]],"date-time":"2024-01-12T11:20:32Z","timestamp":1705058432000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":22,"title":["Use of Technetium\u201099m\u2010Pyrophosphate Single\u2010Photon Emission Computed Tomography\/Computed Tomography in Monitoring Therapeutic Changes of Eplontersen in Patients With Hereditary Transthyretin Amyloid Cardiomyopathy"],"prefix":"10.1161","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5512-9395","authenticated-orcid":false,"given":"An\u2010Li","family":"Yu","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"},{"name":"Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital Hsin\u2010Chu Branch Hsinchu Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi\u2010Chieh","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2859-5117","authenticated-orcid":false,"given":"Cheng\u2010Hsuan","family":"Tsai","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"},{"name":"National Taiwan University College of Medicine Graduate Institute of Clinical Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan\u2010Kun Aden","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6699-2298","authenticated-orcid":false,"given":"Mao\u2010Yuan","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging National Taiwan University Hospital Taipei Taiwan"},{"name":"Department of Medical Imaging and Radiological Technology Yuanpei University of Medical Technology Hsinchu Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chia\u2010Hung","family":"Chou","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0468-4468","authenticated-orcid":false,"given":"Chia\u2010Tung","family":"Shun","sequence":"additional","affiliation":[{"name":"Department of Forensic Medicine and Pathology National Taiwan University Hospital Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4707-1861","authenticated-orcid":false,"given":"Hsueh\u2010Wen","family":"Hsueh","sequence":"additional","affiliation":[{"name":"Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4767-7636","authenticated-orcid":false,"given":"Jimmy Jyh\u2010Ming","family":"Juang","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Division of Cardiology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming\u2010Jen","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ping\u2010Huei","family":"Tseng","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Division of Gastroenterology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3720-9607","authenticated-orcid":false,"given":"Chia\u2010Hua","family":"Hsu","sequence":"additional","affiliation":[{"name":"Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3188-8482","authenticated-orcid":false,"given":"Sung\u2010Tsang","family":"Hsieh","sequence":"additional","affiliation":[{"name":"Department of Neurology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chi\u2010Lun","family":"Ko","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9359-0606","authenticated-orcid":false,"given":"Mei\u2010Fang","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan"},{"name":"Institute of Environmental and Occupational Health Sciences National Taiwan University Taipei 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CARDIO\u2010TTRansform: A Study to Evaluate the Efficacy and Safety of Eplontersen (Formerly Known as ION\u2010682884 IONIS\u2010TTR\u2010LRx and AKCEA\u2010TTR\u2010LRx) in Participants With Transthyretin\u2010Mediated Amyloid Cardiomyopathy (ATTR CM). ClinicalTrials.gov identifier: NCT04136171. Updated August 3 2023. Accessed November 10 2023. https:\/\/classic.clinicaltrials.gov\/ct2\/show\/NCT04136171."},{"key":"e_1_3_2_42_2","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.778"},{"key":"e_1_3_2_43_2","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51328"},{"key":"e_1_3_2_44_2","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2019.1582479"},{"key":"e_1_3_2_45_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12883-021-02243-3"},{"key":"e_1_3_2_46_2","doi-asserted-by":"publisher","DOI":"10.1007\/s40120-022-00414-z"},{"key":"e_1_3_2_47_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.123.015243"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.123.030512","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T12:33:33Z","timestamp":1715603613000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.123.030512"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,16]]},"references-count":46,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,1,16]]}},"alternative-id":["10.1161\/JAHA.123.030512"],"URL":"https:\/\/doi.org\/10.1161\/jaha.123.030512","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,16]]},"assertion":[{"value":"2023-04-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-12-13","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e030512"}}