{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:51:43Z","timestamp":1753876303087,"version":"3.41.2"},"reference-count":15,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2021,8,7]],"date-time":"2021-08-07T00:00:00Z","timestamp":1628294400000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>We report the case of a patient who presented with concomitant aortic valve papillary fibroelastoma (PFE) and cardiac amyloidosis. Although histologically benign, PFE confers an increased thromboembolic risk, and surgical excision is often indicated. However, outcomes of cardiac surgery are poor in patients with cardiac amyloidosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case summary<\/jats:title><jats:p>A 61-year-old man with complaints of dyspnoea and weight loss of 10\u2009kg developing over the past 5 months was evaluated in the cardiology clinic. Echocardiography revealed sessile aortic valve PFE and was also highly suggestive of cardiac amyloidosis. The diagnosis of amyloid light chain amyloidosis secondary to indolent multiple myeloma was eventually confirmed. Therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone allowed full remission over a 6-month period and resulted in marked improvement in symptoms and cardiac function as evaluated by global longitudinal strain. Further workup with cerebral magnetic resonance revealed multiple vascular sequelae. Surgical removal of the aortic fibroelastoma with bioprosthetic aortic valve replacement was performed successfully and the patient had an uneventful recovery.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Papillary fibroelastoma and cardiac amyloidosis are rare and most likely unrelated entities. Concomitant presentation of both conditions in the same patient presents a unique therapeutic challenge. By allowing cardiac function to be monitored during chemotherapy, speckle-tracking echocardiography can prove instrumental in determining the optimal timing of surgical intervention.<\/jats:p><\/jats:sec>","DOI":"10.1093\/ehjcr\/ytab322","type":"journal-article","created":{"date-parts":[[2021,8,7]],"date-time":"2021-08-07T19:00:48Z","timestamp":1628362848000},"source":"Crossref","is-referenced-by-count":0,"title":["Role of imaging for diagnosis and management of aortic valve papillary fibroelastoma and cardiac amyloid light chain amyloidosis: a case report"],"prefix":"10.1093","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9624-9315","authenticated-orcid":false,"given":"Ivan","family":"Dimov","sequence":"first","affiliation":[{"name":"Cardiology Department, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles (ULB), 322 rue Haute, 1000 Brussels, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nathalie","family":"Meuleman","sequence":"additional","affiliation":[{"name":"Hematology Department, Institut Jules Bordet, Universit\u00e9 Libre de Bruxelles (ULB), 121 boulevard de Waterloo, 1000 Brussels, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Didier","family":"de Canni\u00e8re","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Department, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles (ULB), 322 rue Haute, 1000 Brussels, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6154-1546","authenticated-orcid":false,"given":"Philippe","family":"Unger","sequence":"additional","affiliation":[{"name":"Cardiology Department, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles (ULB), 322 rue Haute, 1000 Brussels, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2021,8,7]]},"reference":[{"key":"2021082004250369000_ytab322-B1","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/S0146-2806(99)80001-2","article-title":"Tumors of the heart and pericardium","volume":"24","author":"McAllister","year":"1999","journal-title":"Curr Prob Cardiol"},{"key":"2021082004250369000_ytab322-B2","doi-asserted-by":"crossref","first-page":"2420","DOI":"10.1016\/j.jacc.2015.03.569","article-title":"Prognostic and bioepidemiologic implications of papillary fibroelastomas","volume":"65","author":"Tamin","year":"2015","journal-title":"J Am Coll Cardiol"},{"key":"2021082004250369000_ytab322-B3","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1016\/S0002-8703(03)00249-7","article-title":"Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases","volume":"146","author":"Gowda","year":"2003","journal-title":"Am Heart J"},{"key":"2021082004250369000_ytab322-B4","doi-asserted-by":"crossref","first-page":"2687","DOI":"10.1161\/01.CIR.103.22.2687","article-title":"Clinical and echocardiographic characteristics of papillary fibroelastomas","volume":"103","author":"Sun","year":"2001","journal-title":"Circulation"},{"key":"2021082004250369000_ytab322-B5","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1038\/nrcardio.2017.47","article-title":"Pathology, imaging, and treatment of cardiac tumours","volume":"14","author":"Maleszewski","year":"2017","journal-title":"Nat Rev Cardiol"},{"key":"2021082004250369000_ytab322-B6","doi-asserted-by":"crossref","first-page":"ehab072","DOI":"10.1093\/eurheartj\/ehab072","article-title":"Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases","volume":"42","author":"Garcia-Pavia","year":"2021","journal-title":"Eur Heart J"},{"key":"2021082004250369000_ytab322-B7","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1136\/heartjnl-2012-302353","article-title":"Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis","volume":"98","author":"Phelan","year":"2012","journal-title":"Heart"},{"key":"2021082004250369000_ytab322-B8","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1080\/13506129.2020.1798922","article-title":"Arterial thrombo-embolic events in cardiac amyloidosis: a look beyond atrial fibrillation","volume":"28","author":"Cappelli","year":"2021","journal-title":"Amyloid"},{"key":"2021082004250369000_ytab322-B9","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1007\/s00059-003-2359-1","article-title":"Amyloid of heart and lungs in a patient with low output syndrome after coronary artery bypass grafting","volume":"28","author":"Massoudy","year":"2003","journal-title":"Herz"},{"key":"2021082004250369000_ytab322-B10","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1007\/s11748-012-0164-6","article-title":"An unexpected mortality following cardiac surgery: a post-mortem diagnosis of cardiac amyloidosis","volume":"61","author":"Fitzmaurice","year":"2013","journal-title":"Gen Thorac Cardiovasc Surg"},{"key":"2021082004250369000_ytab322-B11","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1055\/s-2006-924509","article-title":"Cardiac amyloidosis in the cardiosurgical operating room\u2014a rare but fatal trap","volume":"55","author":"Zacek","year":"2007","journal-title":"Thorac Cardiovasc Surg"},{"key":"2021082004250369000_ytab322-B12","doi-asserted-by":"crossref","first-page":"e005066","DOI":"10.1161\/CIRCIMAGING.116.005066","article-title":"Occult transthyretin cardiac amyloid in severe calcific aortic stenosis","volume":"9","author":"Treibel","year":"2018","journal-title":"Circ Cardiovasc Imaging"},{"key":"2021082004250369000_ytab322-B13","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1002\/ejhf.1974","article-title":"Unveiling outcomes in coexisting severe aortic stenosis and transthyretin cardiac amyloidosis","volume":"23","author":"Rosenblum","year":"2021","journal-title":"Eur J Heart Fail"},{"key":"2021082004250369000_ytab322-B14","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/ehjcr\/ytz225","article-title":"Improvement of global longitudinal strain following high-dose chemotherapy and autologous peripheral blood stem cell transplantation in patients with amyloid light-chain cardiac amyloidosis: a case report","volume":"3","author":"Hirata","year":"2019","journal-title":"Eur Heart J Case Rep"},{"key":"2021082004250369000_ytab322-B15","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1016\/j.jcmg.2019.12.014","article-title":"Cardiac amyloidosis mimics, multimodality imaging diagnosis, and treatment","volume":"13","author":"Fazlinezhad","year":"2020","journal-title":"JACC Cardiovasc Imaging"}],"container-title":["European Heart Journal - Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/ehjcr\/advance-article-pdf\/doi\/10.1093\/ehjcr\/ytab322\/39608605\/ytab322.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/ehjcr\/article-pdf\/5\/8\/ytab322\/39810860\/ytab322.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/ehjcr\/article-pdf\/5\/8\/ytab322\/39810860\/ytab322.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,1,6]],"date-time":"2023-01-06T22:00:06Z","timestamp":1673042406000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcr\/article\/doi\/10.1093\/ehjcr\/ytab322\/6345342"}},"subtitle":[],"editor":[{"given":"Nidhi","family":"Madan","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Amir","family":"Khalifa","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Giacomo Tini","family":"Melato","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Nidhish","family":"Tiwari","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Max","family":"Sayers","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Vishal Shahil","family":"Mehta","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2021,8,1]]},"references-count":15,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2021,8,1]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjcr\/ytab322","relation":{},"ISSN":["2514-2119"],"issn-type":[{"type":"electronic","value":"2514-2119"}],"subject":[],"published-other":{"date-parts":[[2021,8,1]]},"published":{"date-parts":[[2021,8,1]]},"article-number":"ytab322"}}