Dia A Smiley, Andrew J Einstein, Kevin J O'Gorman, Denisse Santana, Sergio Teruya, Nicholas Chan, Ani Nalbandian, Timothy J Poterucha, Stephen T Helmke, Akiva Mintz, Kim Goldner, Miroslav Sekulic, Alfonsina Mirabal, Margaret O Cuomo, Samantha Guadalupe, Jeffeny De Los Santos, Mary E Paulino, Kimberly A Mateo, Carlos M Rodriguez, Massiel Jimenez, Abdirahman Wardhere, Dimitrios Bampatsias, Michelle Castillo, Boyu Peng, Mathew S Maurer
{"title":"Early Detection of Transthyretin Cardiac Amyloidosis Using <sup>124</sup>I-Evuzamitide Positron Emission Tomography/Computed Tomography.","authors":"Dia A Smiley, Andrew J Einstein, Kevin J O'Gorman, Denisse Santana, Sergio Teruya, Nicholas Chan, Ani Nalbandian, Timothy J Poterucha, Stephen T Helmke, Akiva Mintz, Kim Goldner, Miroslav Sekulic, Alfonsina Mirabal, Margaret O Cuomo, Samantha Guadalupe, Jeffeny De Los Santos, Mary E Paulino, Kimberly A Mateo, Carlos M Rodriguez, Massiel Jimenez, Abdirahman Wardhere, Dimitrios Bampatsias, Michelle Castillo, Boyu Peng, Mathew S Maurer","doi":"10.1016/j.jcmg.2025.01.018","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Early detection of transthyretin cardiac amyloidosis (ATTR-CA) is vital, because currently available therapies are most effective early in the disease course. Although Perugini grade 2 or 3 uptake on scintigraphy using bone-avid tracers such as <sup>99m</sup>Tc-pyrophosphate is highly specific for ATTR-CA, absent monoclonal proteins, sensitivity is about 70%. Positron emission tomographic (PET)/computed tomographic (CT) imaging using the novel radiolabeled heparan sulfate proteoglycan-binding peptide <sup>124</sup>I-evuzamitide has been shown to be useful for quantifying cardiac amyloid load in systemic amyloidosis.</p><p><strong>Objectives: </strong>The aim of this study was to quantify myocardial <sup>124</sup>I-evuzamitide PET uptake and compare diagnostic performance with that of <sup>99m</sup>Tc-pyrophosphate in patients with ATTR-CA and carriers.</p><p><strong>Methods: </strong>Twenty-five subjects underwent imaging using <sup>124</sup>I-evuzamitide PET/CT imaging: 7 with wild-type ATTR-CA (all Columbia stage I) and 18 transthyretin amyloidosis (ATTR) variant-allele carriers with cardiomyopathy and/or neuropathy. Myocardial uptake was determined as left ventricular percentage of injected dose (mean activity concentration × volume/injected activity) and cardiac amyloid activity (mean specific uptake value × volume) within voxels with uptake above blood pool.</p><p><strong>Results: </strong>Eleven subjects with ATTR and Perugini grade 0 or 1 <sup>99m</sup>Tc-pyrophosphate cardiac scans had cardiac uptake of <sup>124</sup>I-evuzamitide, suggesting that <sup>124</sup>I-evuzamitide PET/CT imaging may detect ATTR amyloid deposits when <sup>99m</sup>Tc-pyrophosphate cardiac scintigraphy does not. <sup>124</sup>I-evuzamitide showed myocardial uptake in all 5 subjects with variant ATTR with biopsy-proven disease and in 2 subjects with wild-type ATTR and negative (grade 0) results on <sup>99m</sup>Tc-pyrophosphate scans but extracardiac biopsies showing ATTR. Myocardial amyloid load was moderately to strongly correlated with disease stage and with echocardiographic and quality-of-life measures.</p><p><strong>Conclusions: </strong><sup>124</sup>I-evuzamitide PET/CT imaging can identify myocardial amyloid in variant transthyretin allele carriers and wild-type subjects when <sup>99m</sup>Tc-pyrophosphate does not. (Evuzamitide in PET/CT to Measure Potential Therapeutic Response in ATTR; NCT05635045).</p>","PeriodicalId":14767,"journal":{"name":"JACC. Cardiovascular imaging","volume":" ","pages":""},"PeriodicalIF":12.8000,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"JACC. Cardiovascular imaging","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.jcmg.2025.01.018","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Early detection of transthyretin cardiac amyloidosis (ATTR-CA) is vital, because currently available therapies are most effective early in the disease course. Although Perugini grade 2 or 3 uptake on scintigraphy using bone-avid tracers such as 99mTc-pyrophosphate is highly specific for ATTR-CA, absent monoclonal proteins, sensitivity is about 70%. Positron emission tomographic (PET)/computed tomographic (CT) imaging using the novel radiolabeled heparan sulfate proteoglycan-binding peptide 124I-evuzamitide has been shown to be useful for quantifying cardiac amyloid load in systemic amyloidosis.
Objectives: The aim of this study was to quantify myocardial 124I-evuzamitide PET uptake and compare diagnostic performance with that of 99mTc-pyrophosphate in patients with ATTR-CA and carriers.
Methods: Twenty-five subjects underwent imaging using 124I-evuzamitide PET/CT imaging: 7 with wild-type ATTR-CA (all Columbia stage I) and 18 transthyretin amyloidosis (ATTR) variant-allele carriers with cardiomyopathy and/or neuropathy. Myocardial uptake was determined as left ventricular percentage of injected dose (mean activity concentration × volume/injected activity) and cardiac amyloid activity (mean specific uptake value × volume) within voxels with uptake above blood pool.
Results: Eleven subjects with ATTR and Perugini grade 0 or 1 99mTc-pyrophosphate cardiac scans had cardiac uptake of 124I-evuzamitide, suggesting that 124I-evuzamitide PET/CT imaging may detect ATTR amyloid deposits when 99mTc-pyrophosphate cardiac scintigraphy does not. 124I-evuzamitide showed myocardial uptake in all 5 subjects with variant ATTR with biopsy-proven disease and in 2 subjects with wild-type ATTR and negative (grade 0) results on 99mTc-pyrophosphate scans but extracardiac biopsies showing ATTR. Myocardial amyloid load was moderately to strongly correlated with disease stage and with echocardiographic and quality-of-life measures.
Conclusions: 124I-evuzamitide PET/CT imaging can identify myocardial amyloid in variant transthyretin allele carriers and wild-type subjects when 99mTc-pyrophosphate does not. (Evuzamitide in PET/CT to Measure Potential Therapeutic Response in ATTR; NCT05635045).
期刊介绍：
JACC: Cardiovascular Imaging, part of the prestigious Journal of the American College of Cardiology (JACC) family, offers readers a comprehensive perspective on all aspects of cardiovascular imaging. This specialist journal covers original clinical research on both non-invasive and invasive imaging techniques, including echocardiography, CT, CMR, nuclear, optical imaging, and cine-angiography.
JACC. Cardiovascular imaging highlights advances in basic science and molecular imaging that are expected to significantly impact clinical practice in the next decade. This influence encompasses improvements in diagnostic performance, enhanced understanding of the pathogenetic basis of diseases, and advancements in therapy.
In addition to cutting-edge research,the content of JACC: Cardiovascular Imaging emphasizes practical aspects for the practicing cardiologist, including advocacy and practice management.The journal also features state-of-the-art reviews, ensuring a well-rounded and insightful resource for professionals in the field of cardiovascular imaging.
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