{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T03:26:41Z","timestamp":1793330801524,"version":"4.5.0"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2021,7,13]],"date-time":"2021-07-13T00:00:00Z","timestamp":1626134400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"BHF Intermediate Clinical Research Fellowship","award":["FS\/18\/21\/33447"],"award-info":[{"award-number":["FS\/18\/21\/33447"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD scintigraphy) is recognized as highly accurate for the non-invasive diagnosis of transthyretin (ATTR) cardiac amyloidosis (CA). A proportion of patients with immunoglobulin light chain (AL) CA have also been reported to show cardiac 99mTc-DPD uptake. Herein, we assessed the frequency and degree of cardiac 99mTc-DPD uptake and its clinical significance among patients with AL CA.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Between 2010 and 2017, 292 consecutive patients with AL CA underwent 99mTc-DPD scintigraphy and were included in this study: 114 (39%) had cardiac 99mTc-DPD uptake: grade 1 in 75%, grade 2 in 17%, and grade 3 in 8% of cases. Patients with cardiac 99mTc-DPD uptake had poorer cardiac systolic function and higher N-terminal pro-brain natriuretic peptide. No differences were noted in cardiac magnetic resonance parameters between patients with and without cardiac 99mTc-DPD uptake (N\u2009=\u200919 and 42, respectively). Patients with cardiac 99mTc-DPD uptake showed a trend to worse survival than those with no uptake (log-rank P\u2009=\u20090.056). Among 22 patients who underwent serial 99mTc-DPD scintigraphy, 5 (23%) showed reduction in the grade of cardiac uptake.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>In this large cohort of patients with AL CA, 99mTc-DPD scintigraphy \u223c40% of cases showed cardiac uptake, including grade 2\u20133 in 10% of all patients (25% of those with cardiac 99mTc-DPD uptake). Cardiac 99mTc-DPD uptake was associated with poorer cardiac function and outcomes. These data highlight the critical importance of ruling out AL amyloidosis in all patients with cardiac 99mTc-DPD uptake to ensure such patients are not assumed to have ATTR CA.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjci\/jeab095","type":"journal-article","created":{"date-parts":[[2021,5,7]],"date-time":"2021-05-07T03:47:57Z","timestamp":1620359277000},"page":"1304-1311","source":"Crossref","is-referenced-by-count":73,"title":["99mTc-DPD scintigraphy in immunoglobulin light chain (AL) cardiac amyloidosis"],"prefix":"10.1093","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0935-2873","authenticated-orcid":false,"given":"Candida Cristina","family":"Quarta","sequence":"first","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"},{"name":"Alexion Pharmaceutical LTD, Stockley Park, 3 Furzeground Way, Hayes, Uxbridge, London UB11 1EZ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4435-2254","authenticated-orcid":false,"given":"Jiexin","family":"Zheng","sequence":"additional","affiliation":[{"name":"Cardiovascular Center, University Hospital of Ferrara, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3466-306X","authenticated-orcid":false,"given":"David","family":"Hutt","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona F","family":"Grigore","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richa","family":"Manwani","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sajitha","family":"Sachchithanantham","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shameem A","family":"Mahmood","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol J","family":"Whelan","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9233-9831","authenticated-orcid":false,"given":"Marianna","family":"Fontana","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Martinez-Naharro","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4524-1436","authenticated-orcid":false,"given":"Liza","family":"Chacko","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen J","family":"Lachmann","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6174-9232","authenticated-orcid":false,"given":"Julian D","family":"Gillmore","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Rapezzi","sequence":"additional","affiliation":[{"name":"Cardiovascular Center, University Hospital of Ferrara, Ferrara, Italy"},{"name":"Maria Cecilia Hospital, GVM Care & Research, Cotignola, RA, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6983-9598","authenticated-orcid":false,"given":"Philip N","family":"Hawkins","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashutosh D","family":"Wechalekar","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,7,13]]},"reference":[{"key":"2021102007542056900_jeab095-B1","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1038\/s41572-018-0034-3","article-title":"Systemic immunoglobulin light chain amyloidosis","volume":"4","author":"Merlini","year":"2018","journal-title":"Nat Rev Dis Primers"},{"key":"2021102007542056900_jeab095-B2","doi-asserted-by":"crossref","first-page":"1567","DOI":"10.1056\/NEJMc1917321","article-title":"Epidemiologic and survival trends in amyloidosis, 1987-2019","volume":"382","author":"Ravichandran","year":"2020","journal-title":"N Engl J Med"},{"key":"2021102007542056900_jeab095-B3","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1016\/j.jacc.2016.06.053","article-title":"AL (light-chain) cardiac amyloidosis: a review of diagnosis and therapy","volume":"68","author":"Falk","year":"2016","journal-title":"J Am Coll Cardiol"},{"key":"2021102007542056900_jeab095-B4","doi-asserted-by":"crossref","first-page":"3751","DOI":"10.1200\/JCO.2004.03.029","article-title":"Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis","volume":"22","author":"Dispenzieri","year":"2004","journal-title":"J Clin Oncol"},{"key":"2021102007542056900_jeab095-B5","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1200\/JCO.2011.38.5724","article-title":"Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements","volume":"30","author":"Kumar","year":"2012","journal-title":"J Clin Oncol"},{"key":"2021102007542056900_jeab095-B6","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1182\/blood-2015-01-620302","article-title":"A European collaborative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis","volume":"126","author":"Palladini","year":"2015","journal-title":"Blood"},{"key":"2021102007542056900_jeab095-B7","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1007\/s00259-010-1642-7","article-title":"Usefulness and limitations of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy in the aetiological diagnosis of amyloidotic cardiomyopathy","volume":"38","author":"Rapezzi","year":"2011","journal-title":"Eur J Nucl Med Mol Imaging"},{"key":"2021102007542056900_jeab095-B8","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1161\/CIRCIMAGING.112.000132","article-title":"(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses","volume":"6","author":"Bokhari","year":"2013","journal-title":"Circ Cardiovasc Imaging"},{"key":"2021102007542056900_jeab095-B9","doi-asserted-by":"crossref","first-page":"2404","DOI":"10.1161\/CIRCULATIONAHA.116.021612","article-title":"Nonbiopsy diagnosis of cardiac transthyretin amyloidosis","volume":"133","author":"Gillmore","year":"2016","journal-title":"Circulation"},{"key":"2021102007542056900_jeab095-B10","first-page":"42","article-title":"Imaging of cardiac amyloidosis by (99m)Tc-PYP scintigraphy","volume":"18","author":"Papantoniou","year":"2015","journal-title":"Hell J Nucl Med"},{"key":"2021102007542056900_jeab095-B11","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1056\/NEJM199008233230803","article-title":"Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component","volume":"323","author":"Hawkins","year":"1990","journal-title":"N Engl J Med"},{"key":"2021102007542056900_jeab095-B12","doi-asserted-by":"crossref","first-page":"E2403","DOI":"10.1002\/humu.22619","article-title":"Online registry for mutations in hereditary amyloidosis including nomenclature recommendations","volume":"35","author":"Rowczenio","year":"2014","journal-title":"Hum Mutat"},{"key":"2021102007542056900_jeab095-B13","doi-asserted-by":"crossref","first-page":"4541","DOI":"10.1200\/JCO.2011.37.7614","article-title":"New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes","volume":"30","author":"Palladini","year":"2012","journal-title":"J Clin Oncol"},{"key":"2021102007542056900_jeab095-B14","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1080\/13506120802676914","article-title":"Biochemical characterisation of amyloid by endomyocardial biopsy","volume":"16","author":"Benson","year":"2009","journal-title":"Amyloid"},{"key":"2021102007542056900_jeab095-B15","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1002\/ajh.20381","article-title":"Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th international symposium on amyloid and amyloidosis","volume":"79","author":"Gertz","year":"2005","journal-title":"Am J Hematol"},{"key":"2021102007542056900_jeab095-B16","doi-asserted-by":"crossref","first-page":"1786","DOI":"10.1056\/NEJMoa013354","article-title":"Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis","volume":"346","author":"Lachmann","year":"2002","journal-title":"N Engl J Med"},{"key":"2021102007542056900_jeab095-B17","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1016\/j.cardfail.2019.08.002","article-title":"ASNC\/AHA\/ASE\/EANM\/HFSA\/ISA\/SCMR\/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: part 2 of 2-diagnostic criteria and appropriate utilization","volume":"25","author":"Dorbala","year":"2019","journal-title":"J Card Fail"},{"key":"2021102007542056900_jeab095-B18","doi-asserted-by":"crossref","first-page":"1440","DOI":"10.1016\/j.echo.2005.10.005","article-title":"Recommendations for chamber quantification: a report from the American Society of Echocardiography\u2019s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology","volume":"18","author":"Lang","year":"2005","journal-title":"J Am Soc Echocardiogr"},{"key":"2021102007542056900_jeab095-B19","doi-asserted-by":"crossref","first-page":"1840","DOI":"10.1161\/CIRCULATIONAHA.113.006242","article-title":"Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis","volume":"129","author":"Quarta","year":"2014","journal-title":"Circulation"},{"key":"2021102007542056900_jeab095-B20","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1016\/j.jacc.2017.05.053","article-title":"Magnetic resonance in transthyretin cardiac amyloidosis","volume":"70","author":"Martinez-Naharro","year":"2017","journal-title":"J Am Coll Cardiol"},{"key":"2021102007542056900_jeab095-B21","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1016\/j.jcmg.2018.02.006","article-title":"Native T1 and extracellular volume in transthyretin amyloidosis","volume":"12","author":"Martinez-Naharro","year":"2019","journal-title":"JACC Cardiovasc Imaging"},{"key":"2021102007542056900_jeab095-B22","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1093\/ehjci\/jeu107","article-title":"Utility and limitations of 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy in systemic amyloidosis","volume":"15","author":"Hutt","year":"2014","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"2021102007542056900_jeab095-B23","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1016\/j.jacc.2005.05.073","article-title":"Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy","volume":"46","author":"Perugini","year":"2005","journal-title":"J Am Coll Cardiol"},{"key":"2021102007542056900_jeab095-B24","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/j.jcmg.2011.03.016","article-title":"Role of (99m)Tc-DPD scintigraphy in diagnosis and prognosis of hereditary transthyretin-related cardiac amyloidosis","volume":"4","author":"Rapezzi","year":"2011","journal-title":"JACC Cardiovasc Imaging"},{"key":"2021102007542056900_jeab095-B25","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.carpath.2016.07.001","article-title":"Varying levels of small microcalcifications and macrophages in ATTR and AL cardiac amyloidosis: implications for utilizing nuclear medicine studies to subtype amyloidosis","volume":"25","author":"Stats","year":"2016","journal-title":"Cardiovasc Pathol"},{"key":"2021102007542056900_jeab095-B26","doi-asserted-by":"crossref","first-page":"1008","DOI":"10.1161\/01.RES.0000126569.75419.74","article-title":"Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress","volume":"94","author":"Brenner","year":"2004","journal-title":"Circ Res"},{"key":"2021102007542056900_jeab095-B27","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1093\/ejechocard\/jer289","article-title":"Right ventricular function in AL amyloidosis: characteristics and prognostic implication","volume":"13","author":"Cappelli","year":"2012","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"2021102007542056900_jeab095-B28","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1016\/j.jcmg.2018.02.016","article-title":"Cardiac structural and functional consequences of amyloid deposition by cardiac magnetic resonance and echocardiography and their prognostic roles","volume":"12","author":"Knight","year":"2019","journal-title":"JACC Cardiovasc Imaging"},{"key":"2021102007542056900_jeab095-B29","doi-asserted-by":"crossref","first-page":"2111","DOI":"10.1182\/blood-2016-11-751628","article-title":"Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death","volume":"129","author":"Muchtar","year":"2017","journal-title":"Blood"}],"container-title":["European Heart Journal - Cardiovascular Imaging"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/ehjcimaging\/article-pdf\/22\/11\/1304\/40759288\/jeab095.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/ehjcimaging\/article-pdf\/22\/11\/1304\/40759288\/jeab095.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T00:12:42Z","timestamp":1634775162000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcimaging\/article\/22\/11\/1304\/6319841"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,13]]},"references-count":29,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2021,7,13]]},"published-print":{"date-parts":[[2021,10,19]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjci\/jeab095","relation":{},"ISSN":["2047-2404","2047-2412"],"issn-type":[{"value":"2047-2404","type":"print"},{"value":"2047-2412","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,11,1]]},"published":{"date-parts":[[2021,7,13]]}}}