{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T04:09:01Z","timestamp":1788926941384,"version":"build-2803163510"},"reference-count":58,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T00:00:00Z","timestamp":1639094400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000765","name":"University College London","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:p>Systemic amyloidosis is a rare, heterogenous group of diseases characterized by extracellular infiltration and deposition of amyloid fibrils. Cardiac amyloidosis (CA) occurs when these fibrils deposit within the myocardium. Untreated, this inevitably leads to progressive heart failure and fatality. Historically, treatment has remained supportive, however, there are now targeted disease-modifying therapeutics available to patients with CA. Advances in echocardiography, cardiac magnetic resonance (CMR) and repurposed bone scintigraphy have led to a surge in diagnoses of CA and diagnosis at an earlier stage of the disease natural history. CMR has inherent advantages in tissue characterization which has allowed us to better understand the pathological disease process behind CA. Combined with specialist assessment and repurposed bone scintigraphy, diagnosis of CA can be made without the need for invasive histology in a significant proportion of patients. With existing targeted therapeutics, and novel agents being developed, understanding these imaging modalities is crucial to achieving early diagnosis for patients with CA. This will allow for early treatment intervention, accurate monitoring of disease course over time, and thereby improve the length and quality of life of patients with a disease that historically had an extremely poor prognosis. In this review, we discuss key radiological features of CA, focusing on the two most common types; immunoglobulin light chain (AL) and transthyretin (ATTR) CA. We highlight recent advances in imaging techniques particularly in respect of their clinical application and utility in diagnosis of CA as well as for tracking disease change over time.<\/jats:p>","DOI":"10.3389\/fcvm.2021.751293","type":"journal-article","created":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T00:58:31Z","timestamp":1639097911000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":42,"title":["Cardiac Amyloidosis: A Review of Current Imaging Techniques"],"prefix":"10.3389","volume":"8","author":[{"given":"Yousuf","family":"Razvi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rishi K.","family":"Patel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianna","family":"Fontana","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian D.","family":"Gillmore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,12,10]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1016\/j.coph.2005.10.005","article-title":"Systemic amyloidosis","volume":"6","author":"Lachmann","year":"2006","journal-title":"Curr Opin Pharmacol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"2641","DOI":"10.1016\/S0140-6736(15)01274-X","article-title":"Systemic amyloidosis","volume":"387","author":"Wechalekar","year":"2016","journal-title":"Lancet."},{"key":"B3","doi-asserted-by":"publisher","first-page":"1180","DOI":"10.1007\/s11886-019-1180-2","article-title":"Cardiac amyloidosis: updates in imaging","volume":"21","author":"Chacko","year":"2019","journal-title":"Curr Cardiol Rep."},{"key":"B4","doi-asserted-by":"publisher","first-page":"s30","DOI":"10.7861\/clinmedicine.18-2-s30","article-title":"Cardiac amyloidosis","volume":"18","author":"Martinez-Naharro","year":"2018","journal-title":"Clin Med J R College Phys London."},{"key":"B5","doi-asserted-by":"publisher","first-page":"388","DOI":"10.1148\/radiol.2015141744","article-title":"Differential myocyte responses in patients with cardiac transthyretin amyloidosis and light-chain amyloidosis: A cardiac MR imaging study","volume":"277","author":"Fontana","year":"2015","journal-title":"Radiology."},{"key":"B6","doi-asserted-by":"publisher","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 College Cardiol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1161\/CIRCULATIONAHA.118.038169","article-title":"Natural history, quality of life, and outcome in cardiac transthyretin amyloidosis","volume":"140","author":"Lane","year":"2019","journal-title":"Circulation."},{"key":"B8","doi-asserted-by":"publisher","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":"B9","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1056\/NEJMoa1716793","article-title":"Inotersen treatment for patients with hereditary transthyretin amyloidosis","volume":"379","author":"Benson","year":"2018","journal-title":"N Engl J Med."},{"key":"B10","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1056\/NEJMe1805499","article-title":"Oligonucleotide drugs for transthyretin amyloidosis","volume":"379","author":"Buxbaum","year":"2018","journal-title":"N Engl J Med."},{"key":"B11","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1056\/NEJMoa1716153","article-title":"Patisiran, an RNAi therapeutic, for hereditary transthyretin amyloidosis","volume":"379","author":"Adams","year":"2018","journal-title":"N Engl J Med."},{"key":"B12","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.ijcard.2018.05.018","article-title":"Therapies for cardiac light chain amyloidosis: An update","volume":"271","author":"Aimo","year":"2018","journal-title":"Int J Cardiol."},{"key":"B13","doi-asserted-by":"publisher","first-page":"2919","DOI":"10.1016\/j.jacc.2018.03.536","article-title":"Myocardial edema and prognosis in amyloidosis","volume":"71","author":"Kotecha","year":"2018","journal-title":"J Am College Cardiol."},{"key":"B14","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1016\/j.hlc.2019.11.019","article-title":"Amyloid cardiomyopathy","volume":"29","author":"Bart","year":"2020","journal-title":"Heart Lung Circulation."},{"key":"B15","doi-asserted-by":"publisher","first-page":"1065","DOI":"10.1136\/heartjnl-2016-310704","article-title":"Light-chain cardiac amyloidosis: Strategies to promote early diagnosis and cardiac response","volume":"103","author":"Grogan","year":"2017","journal-title":"Heart."},{"key":"B16","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1111\/bjh.13805","article-title":"Recent advances in the management of AL Amyloidosis","volume":"172","author":"Kastritis","year":"2016","journal-title":"Br J Haematol."},{"key":"B17","doi-asserted-by":"publisher","first-page":"2585","DOI":"10.1093\/eurheartj\/ehv338","article-title":"Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction","volume":"36","author":"Gonz\u00e1lez-L\u00f3pez","year":"2015","journal-title":"Eur Heart J."},{"key":"B18","doi-asserted-by":"publisher","first-page":"2879","DOI":"10.1093\/eurheartj\/ehx350","article-title":"Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement","volume":"38","author":"Castano","year":"2017","journal-title":"Eur Heart J."},{"key":"B19","doi-asserted-by":"publisher","first-page":"68","DOI":"10.3109\/13506129.2012.674074","article-title":"Senile systemic amyloidosis: Are regional differences real or do they reflect different diagnostic suspicion and use of techniques","volume":"19","author":"Falk","year":"2012","journal-title":"Amyloid"},{"key":"B20","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1080\/07853890701842988","article-title":"Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau : A population-based autopsy study","volume":"40","author":"Tanskanen","year":"2008","journal-title":"Ann Med."},{"key":"B21","doi-asserted-by":"publisher","first-page":"1286","DOI":"10.1161\/CIRCULATIONAHA.111.078915","article-title":"Transthyretin (TTR) cardiac amyloidosis","volume":"126","author":"Ruberg","year":"2012","journal-title":"Circulation."},{"key":"B22","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1185\/03007995.2012.754348","article-title":"THAOS-the transthyretin amyloidosis outcomes survey: initial report on clinical manifestations in patients with hereditary and wild-type transthyretin amyloidosis","volume":"29","author":"Coelho","year":"2013","journal-title":"Curr Med Res Opin."},{"key":"B23","doi-asserted-by":"publisher","first-page":"160","DOI":"10.3109\/13506120308998998","article-title":"Tabulation of human transthyretin (TTR) variants, 2003","volume":"10","author":"Connors","year":"2003","journal-title":"Amyloid."},{"key":"B24","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1016\/j.ahj.2009.08.006","article-title":"Cardiac amyloidosis in African Americans: Comparison of clinical and laboratory features of transthyretin V122I amyloidosis and immunoglobulin light chain amyloidosis","volume":"158","author":"Connors","year":"2009","journal-title":"Am Heart J."},{"key":"B25","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1007\/s10741-014-9462-7","article-title":"Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs","volume":"20","author":"Casta\u00f1o","year":"2015","journal-title":"Heart Failure Rev."},{"key":"B26","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1111\/anec.12036","article-title":"Diagnostic and prognostic value of low QRS voltages in cardiac AL amyloidosis","volume":"18","author":"Mussinelli","year":"2013","journal-title":"Ann Noninvasive Electrocardiol."},{"key":"B27","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1016\/j.ahj.2012.04.013","article-title":"The electrocardiographic features associated with cardiac amyloidosis of variant transthyretin isoleucine 122 type in Afro-Caribbean patients","volume":"164","author":"Dungu","year":"2012","journal-title":"Am Heart J."},{"key":"B28","doi-asserted-by":"publisher","first-page":"748","DOI":"10.1136\/heartjnl-2015-308657","article-title":"Prognostic implication of relative regional strain ratio in cardiac amyloidosis","volume":"102","author":"Senapati","year":"2016","journal-title":"Heart."},{"key":"B29","doi-asserted-by":"publisher","first-page":"1","DOI":"10.20944\/preprints202009.0575.v1","article-title":"Right heart changes impact on clinical phenotype of amyloid cardiac involvement: A single centre study","volume":"10","author":"Cicco","year":"2020","journal-title":"Life."},{"key":"B30","doi-asserted-by":"publisher","first-page":"45","DOI":"10.3109\/13506129.2014.992515","article-title":"Prognostic significance of semiautomatic quantification of left ventricular long axis shortening in systemic light-chain amyloidosis","volume":"22","author":"Riffel","year":"2015","journal-title":"Amyloid."},{"key":"B31","doi-asserted-by":"publisher","first-page":"1439","DOI":"10.1093\/eurheartj\/ehz905","article-title":"Echocardiographic phenotype and prognosis in transthyretin cardiac amyloidosis","volume":"41","author":"Chacko","year":"2020","journal-title":"Eur Heart J."},{"key":"B32","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1093\/eurheartj\/ehi040","article-title":"Left atrial myopathy in cardiac amyloidosis: Implications of novel echocardiographic techniques","volume":"26","author":"Modesto","year":"2005","journal-title":"Eur Heart J."},{"key":"B33","doi-asserted-by":"publisher","first-page":"2268","DOI":"10.1016\/j.jcmg.2021.06.022","article-title":"Clinical importance of left atrial infiltration in cardiac transthyretin amyloidosis","volume":"14","author":"Bandera","year":"2021","journal-title":"JACC: Cardiovasc Imaging"},{"key":"B34","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1016\/j.jacc.2020.11.006","article-title":"Prevalence and outcomes of concomitant aortic stenosis and cardiac amyloidosis","volume":"77","author":"Nitsche","year":"2021","journal-title":"J Am College Cardiol."},{"key":"B35","doi-asserted-by":"publisher","first-page":"2420","DOI":"10.1161\/CIRCULATIONAHA.107.697763","article-title":"Intracardiac thrombosis and embolism in patients with cardiac amyloidosis","volume":"116","author":"Feng","year":"2007","journal-title":"Circulation."},{"key":"B36","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1007\/s10741-014-9466-3","article-title":"Echocardiography in cardiac amyloidosis","volume":"20","author":"Falk","year":"2015","journal-title":"Heart Failure Rev."},{"key":"B37","doi-asserted-by":"publisher","first-page":"909","DOI":"10.1016\/j.jcmg.2019.10.011","article-title":"Multiparametric echocardiography scores for the diagnosis of cardiac amyloidosis","volume":"13","author":"Boldrini","year":"2020","journal-title":"JACC: Cardiovascular Imaging."},{"key":"B38","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1161\/01.CIR.0000152819.97857.9D","article-title":"Cardiovascular magnetic resonance in cardiac amyloidosis","volume":"111","author":"Maceira","year":"2005","journal-title":"Circulation."},{"key":"B39","doi-asserted-by":"publisher","first-page":"1267","DOI":"10.1016\/j.jcmg.2016.01.036","article-title":"Prognostic value of late gadolinium enhancement CMR in systemic amyloidosis","volume":"9","author":"Raina","year":"2016","journal-title":"JACC: Cardiovascular Imaging."},{"key":"B40","doi-asserted-by":"publisher","first-page":"1278","DOI":"10.1016\/j.jcmg.2016.02.035","article-title":"Staging cardiac amyloidosis with CMR: understanding the different phenotypes","volume":"9","author":"Fontana","year":"2016","journal-title":"JACC: Cardiovascular Imaging."},{"key":"B41","doi-asserted-by":"publisher","first-page":"772","DOI":"10.1148\/radiol.15150025","article-title":"Intracranial gadolinium deposition after contrast-enhanced MR imaging","volume":"275","author":"McDonald","year":"2015","journal-title":"Radiology."},{"key":"B42","doi-asserted-by":"publisher","first-page":"488","DOI":"10.1016\/j.jcmg.2012.11.013","article-title":"Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis","volume":"6","author":"Karamitsos","year":"2013","journal-title":"JACC: Cardiovascular Imaging."},{"key":"B43","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1016\/j.jcmg.2013.10.008","article-title":"Native T1 mapping in transthyretin amyloidosis","volume":"7","author":"Fontana","year":"2014","journal-title":"JACC: Cardiovascular Imaging."},{"key":"B44","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1161\/CIRCIMAGING.112.978627","article-title":"Quantification of myocardial extracellular volume fraction in systemic AL amyloidosis: An equilibrium contrast cardiovascular magnetic resonance study","volume":"6","author":"Banypersad","year":"2013","journal-title":"Circulation."},{"key":"B45","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.jcmg.2017.02.012","article-title":"CMR-verified regression of cardiac AL amyloid after chemotherapy","volume":"11","author":"Martinez-Naharro","year":"2018","journal-title":"JACC: Cardiovascular Imaging."},{"key":"B46","doi-asserted-by":"publisher","first-page":"CIRCIMAGING","DOI":"10.1161\/CIRCIMAGING.121.012506","article-title":"Cardiac magnetic resonance-derived extracellular volume mapping for the quantification of hepatic and splenic amyloid","volume":"14","author":"Chacko","year":"2021","journal-title":"Circulation."},{"key":"B47","doi-asserted-by":"publisher","first-page":"355.e15","DOI":"10.1016\/j.amjmed.2005.08.043","article-title":"Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis","volume":"119","author":"Hazenberg","year":"2006","journal-title":"Am J Med."},{"key":"B48","doi-asserted-by":"publisher","first-page":"102","DOI":"10.1161\/CIRCIMAGING.111.967836","article-title":"Improved detection of myocardial involvement in acute inflammatory cardiomyopathies using T2 mapping","volume":"5","author":"Thavendiranathan","year":"2012","journal-title":"Circulation"},{"key":"B49","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1097\/RTI.0000000000000077","article-title":"Myocardial tissue characterization by magnetic resonance imaging: Novel applications of T1 and T2 mapping","volume":"29","author":"Ferreira","year":"2014","journal-title":"J Thoracic Imaging."},{"key":"B50","doi-asserted-by":"publisher","first-page":"826","DOI":"10.1016\/S0002-9149(83)80140-4","article-title":"Sensitivity of technetium-99m-pyrophosphate scintigraphy in diagnosing cardiac amyloidosis","volume":"51","author":"Falk","year":"1983","journal-title":"Am J Cardiol."},{"key":"B51","doi-asserted-by":"publisher","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 College Cardiol."},{"key":"B52","doi-asserted-by":"publisher","first-page":"1344","DOI":"10.1093\/ehjci\/jew325","article-title":"Prognostic utility of the Perugini grading of 99mTc-DPD scintigraphy in transthyretin (ATTR) amyloidosis and its relationship with skeletal muscle and soft tissue amyloid","volume":"18","author":"Hutt","year":"2017","journal-title":"Eur Heart J."},{"key":"B53","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1056\/NEJMoa2107454","article-title":"CRISPR-Cas9 in vivo gene editing for transthyretin amyloidosis","volume":"385","author":"Gillmore","year":"2021","journal-title":"N Engl J Med."},{"key":"B54","doi-asserted-by":"publisher","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":"B55","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1038\/s41408-018-0080-9","article-title":"Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2018","volume":"8","author":"Gertz","year":"2018","journal-title":"Blood Cancer J"},{"key":"B56","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1016\/j.tcm.2017.07.004","article-title":"Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment","volume":"28","author":"Siddiqi","year":"2018","journal-title":"Trends Cardiovas Med"},{"key":"B57","doi-asserted-by":"publisher","first-page":"P3","DOI":"10.1186\/1750-1172-10-S1-P3","article-title":"The role of complement in ATTR amyloidosis: a new therapeutic avenue?","volume":"10","author":"Panayiotou","year":"2015","journal-title":"Orphanet J Rare Dis"},{"key":"B58","doi-asserted-by":"publisher","first-page":"1007","DOI":"10.1056\/NEJMoa1805689","article-title":"Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy","volume":"379","author":"Maurer","year":"2018","journal-title":"N Engl J Med."}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2021.751293\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T00:58:33Z","timestamp":1639097913000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2021.751293\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,10]]},"references-count":58,"alternative-id":["10.3389\/fcvm.2021.751293"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2021.751293","relation":{},"ISSN":["2297-055X"],"issn-type":[{"value":"2297-055X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12,10]]},"article-number":"751293"}}