{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T22:46:25Z","timestamp":1790721985937,"version":"4.1.0"},"reference-count":35,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2023,3,22]],"date-time":"2023-03-22T00:00:00Z","timestamp":1679443200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,3,22]],"date-time":"2023-03-22T00:00:00Z","timestamp":1679443200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Radiol med"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Purpose<\/jats:title>\n                <jats:p>Erdheim\u2013Chester disease (ECD) is a rare multisystem histiocytosis, whose cardiovascular involvement has not been systematically characterized so far. We aimed to systematically (qualitatively and quantitatively) describe the features of cardiovascular involvement in a large cohort of ECD patients and to evaluate its impact on myocardial fibrosis extension and cardiac function.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Material and methods<\/jats:title>\n                <jats:p>Among 54 patients with biopsy-proven ECD, 29 patients (59\u2009\u00b1\u200912\u00a0years, 79% males) underwent 1.5-T CMR using a standardized protocol for qualitative and quantitative assessment of disease localization, evaluation of atrial and ventricular function, and assessment of non-dense and dense myocardial fibrosis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>The right atrioventricular (AV) groove was the most commonly affected cardiac site (76%) followed by the right atrial walls (63%), thoracic aorta (59%), and superior vena cava (38%). Right AV groove involvement, encasing the right ventricular artery, was associated with non-dense myocardial fibrosis in the infero-septal (20\/26 patients) and the inferior (14\/26 patients) mid-basal left ventricular (LV) wall. In two patients with right AV groove localization, LGE revealed myocardial infarction in the same myocardial segments. Three out of five patients with left AV groove involvement had non-dense LGE on the lateral LV mid-basal wall. Bulky right atrial pseudomass was associated with atrial dysfunction and superior and inferior vena cava stenosis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>In ECD patients, AV groove localization is associated with LV wall fibrosis in the downstream coronary territories, suggesting hemodynamic alterations due to coronary encasement. Conversely, atrial pseudomass ECD localizations impact on atrial contractility causing atrial dysfunction and are associated with atrio-caval junction stenosis.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s11547-023-01616-7","type":"journal-article","created":{"date-parts":[[2023,3,22]],"date-time":"2023-03-22T14:06:14Z","timestamp":1679493974000},"page":"456-466","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Cardiovascular involvement in Erdheim\u2013Chester diseases is associated with myocardial fibrosis and atrial dysfunction"],"prefix":"10.1007","volume":"128","author":[{"given":"Anna","family":"Palmisano","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corrado","family":"Campochiaro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Vignale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Tomelleri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giacomo","family":"De Luca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Bruno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caterina B.","family":"Monti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulio","family":"Cavalli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Dagna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1170-6266","authenticated-orcid":false,"given":"Antonio","family":"Esposito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,3,22]]},"reference":[{"key":"1616_CR1","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1016\/j.ejim.2015.03.004","volume":"26","author":"C Campochiaro","year":"2015","unstructured":"Campochiaro C, Tomelleri A, Cavalli G et al (2015) Erdheim-Chester disease. Eur J Intern Med 26:223\u2013229","journal-title":"Eur J Intern Med"},{"key":"1616_CR2","doi-asserted-by":"publisher","first-page":"1929","DOI":"10.1182\/blood.2019003507","volume":"135","author":"G Goyal","year":"2020","unstructured":"Goyal G, Heaney ML, Collin M et al (2020) Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood 135:1929\u20131945","journal-title":"Blood"},{"key":"1616_CR3","doi-asserted-by":"publisher","first-page":"2778","DOI":"10.1182\/blood-2010-06-294108","volume":"117","author":"L Arnaud","year":"2011","unstructured":"Arnaud L, Hervier B, N\u00e9el A et al (2011) CNS involvement and treatment with interferon-\u03b1 are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood 117:2778\u20132782","journal-title":"Blood"},{"key":"1616_CR4","doi-asserted-by":"publisher","first-page":"e597","DOI":"10.1161\/CIRCULATIONAHA.108.825075","volume":"119","author":"J Haroche","year":"2009","unstructured":"Haroche J, Cluzel P, Toledano D et al (2009) Cardiac involvement in Erdheim-Chester disease. Circulation 119:e597\u2013e598","journal-title":"Circulation"},{"key":"1616_CR5","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000001365","volume":"94","author":"MA Nicolazzi","year":"2015","unstructured":"Nicolazzi MA, Carnicelli A, Fuorlo M et al (2015) Cardiovascular involvement in Erdheim-Chester disease. Medicine 94:e1365","journal-title":"Medicine"},{"key":"1616_CR6","doi-asserted-by":"publisher","first-page":"205846011559227","DOI":"10.1177\/2058460115592273","volume":"4","author":"A Ponsiglione","year":"2015","unstructured":"Ponsiglione A, Puglia M, Barbuto L et al (2015) Cardiac involvement in Erdheim-Chester disease: MRI findings and literature revision. Acta Radiol Open 4:205846011559227","journal-title":"Acta Radiol Open"},{"key":"1616_CR7","doi-asserted-by":"publisher","first-page":"2468","DOI":"10.1182\/blood-2016-07-724815","volume":"128","author":"D Gianfreda","year":"2016","unstructured":"Gianfreda D, Palumbo AA, Rossi E et al (2016) Cardiac involvement in Erdheim-Chester disease: an MRI study. Blood 128:2468\u20132471","journal-title":"Blood"},{"key":"1616_CR8","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1186\/1750-1172-8-137","volume":"8","author":"RD Mazor","year":"2013","unstructured":"Mazor RD, Manevich-Mazor M, Shoenfeld Y (2013) Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis 8:137","journal-title":"Orphanet J Rare Dis"},{"key":"1616_CR9","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.118.008531","volume":"11","author":"T Quinaglia-Silva","year":"2018","unstructured":"Quinaglia-Silva T, Medina F, Ramos CD, Coelho-Filho OR (2018) Cardiac involvement in Erdheim-Chester disease. Circ Cardiovasc Imaging 11:e008531","journal-title":"Circ Cardiovasc Imaging"},{"key":"1616_CR10","doi-asserted-by":"publisher","first-page":"7019","DOI":"10.1200\/JCO.2019.37.15_suppl.7019","volume":"37","author":"M Bravetti","year":"2019","unstructured":"Bravetti M, Azoulay L-D, Cohen-Aubart F et al (2019) Correlation of BRAF V600E mutation with cardiac involvement assessed by heart imaging in a monocentric series of 205 patients with Erdheim-Chester disease. J Clin Oncol 37:7019\u20137019","journal-title":"J Clin Oncol"},{"key":"1616_CR11","doi-asserted-by":"publisher","first-page":"229","DOI":"10.1111\/echo.14231","volume":"36","author":"AS Ghotra","year":"2019","unstructured":"Ghotra AS, Thompson K, Lopez-Mattei J et al (2019) Cardiovascular manifestations of Erdheim-Chester disease. Echocardiography 36:229\u2013236","journal-title":"Echocardiography"},{"key":"1616_CR12","doi-asserted-by":"publisher","first-page":"20190473","DOI":"10.1259\/bjr.20190473","volume":"92","author":"JP Das","year":"2019","unstructured":"Das JP, Xie L, Riedl CC et al (2019) Cardiothoracic manifestations of Erdheim-Chester disease. Br J Radiol 92:20190473","journal-title":"Br J Radiol"},{"key":"1616_CR13","doi-asserted-by":"publisher","first-page":"752","DOI":"10.1007\/s11547-012-0899-2","volume":"118","author":"E di Cesare","year":"2013","unstructured":"di Cesare E, Cademartiri F, Carbone I et al (2013) Clinical indications for the use of cardiac MRI. By the SIRM Study Group on Cardiac Imaging. Radiol Med 118:752\u2013798","journal-title":"Radiol Med"},{"key":"1616_CR14","doi-asserted-by":"publisher","first-page":"1013","DOI":"10.1007\/s11547-020-01286-9","volume":"125","author":"A Esposito","year":"2020","unstructured":"Esposito A, Gallone G, Palmisano A et al (2020) The current landscape of imaging recommendations in cardiovascular clinical guidelines: toward an imaging-guided precision medicine. Radiol Med 125:1013\u20131023","journal-title":"Radiol Med"},{"key":"1616_CR15","doi-asserted-by":"publisher","first-page":"515","DOI":"10.2459\/JCM.0000000000001170","volume":"22","author":"M Francone","year":"2021","unstructured":"Francone M, Aquaro GD, Barison A et al (2021) Appropriate use criteria for cardiovascular MRI: SIC \u2013 SIRM position paper Part 2 (myocarditis, pericardial disease, cardiomyopathies and valvular heart disease). J Cardiovasc Med 22:515\u2013529","journal-title":"J Cardiovasc Med"},{"key":"1616_CR16","doi-asserted-by":"publisher","first-page":"e11","DOI":"10.1136\/annrheumdis-2019-216610","volume":"81","author":"C Campochiaro","year":"2022","unstructured":"Campochiaro C, Cavalli G, Farina N et al (2022) Efficacy and improved tolerability of combination therapy with interleukin-1 blockade and MAPK pathway inhibitors for the treatment of Erdheim-Chester disease. Ann Rheum Dis 81:e11\u2013e11","journal-title":"Ann Rheum Dis"},{"issue":"3","key":"1616_CR17","doi-asserted-by":"publisher","first-page":"243","DOI":"10.1080\/03009742.2021.1992846","volume":"51","author":"C Campochiaro","year":"2021","unstructured":"Campochiaro C, Tomelleri A, Farina N et al (2021) Myocarditis as a manifestation of Erdheim-Chester disease: successful use of anti- IL1 and BRAF inhibitor combination therapy. Scand J Rheumatol 51(3):243\u2013245","journal-title":"Scand J Rheumatol"},{"key":"1616_CR18","doi-asserted-by":"publisher","first-page":"986","DOI":"10.1093\/rheumatology\/keaa430","volume":"60","author":"R Cui","year":"2021","unstructured":"Cui R, Chen M, Dai S-M (2021) Coated aorta in Erdheim-Chester disease. Rheumatology 60:986\u2013987","journal-title":"Rheumatology"},{"issue":"1","key":"1616_CR19","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1186\/s12968-016-0284-8","volume":"18","author":"AM Maceira","year":"2016","unstructured":"Maceira AM, Cosin-Sales J, Prasad SK, Pennell DJ (2016) Characterization of left and right atrial function in healthy volunteers by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 18(1):64","journal-title":"J Cardiovasc Magn Reson"},{"key":"1616_CR20","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1097\/00005792-199605000-00005","volume":"75","author":"C Veyssier-Belot","year":"1996","unstructured":"Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D et al (1996) Erdheim-Chester disease clinical and radiologic characteristics of 59 cases. Medicine 75:157\u2013169","journal-title":"Medicine"},{"key":"1616_CR21","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1097\/01.md.0000145368.17934.91","volume":"83","author":"J Haroche","year":"2004","unstructured":"Haroche J, Amoura Z, Dion E et al (2004) Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease. Medicine 83:371\u2013392","journal-title":"Medicine"},{"key":"1616_CR22","doi-asserted-by":"publisher","first-page":"375","DOI":"10.1097\/PAT.0b013e32834685d5","volume":"43","author":"P Vaideeswar","year":"2011","unstructured":"Vaideeswar P, Vaz WF (2011) Erdheim-Chester disease with extensive coronary arterial involvement. Pathology 43:375\u2013378","journal-title":"Pathology"},{"key":"1616_CR23","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1093\/ehjci\/jeaa023","volume":"21","author":"CH Nomura","year":"2020","unstructured":"Nomura CH, Assuncao-Jr AN, Guimar\u00e3es PO et al (2020) Association between perivascular inflammation and downstream myocardial perfusion in patients with suspected coronary artery disease. Eur Heart J Cardiovasc Imaging 21:599\u2013605","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"1616_CR24","doi-asserted-by":"publisher","first-page":"4018","DOI":"10.1002\/art.22280","volume":"54","author":"A Stoppacciaro","year":"2006","unstructured":"Stoppacciaro A, Ferrarini M, Salmaggi C et al (2006) Immunohistochemical evidence of a cytokine and chemokine network in three patients with Erdheim-Chester disease: Implications for pathogenesis. Arthritis Rheum 54:4018\u20134022","journal-title":"Arthritis Rheum"},{"key":"1616_CR25","doi-asserted-by":"publisher","first-page":"1203","DOI":"10.1093\/rheumatology\/kep461","volume":"49","author":"L Dagna","year":"2010","unstructured":"Dagna L, Girlanda S, Langheim S et al (2010) Erdheim-Chester disease: report on a case and new insights on its immunopathogenesis. Rheumatology 49:1203\u20131206","journal-title":"Rheumatology"},{"key":"1616_CR26","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0059308","volume":"8","author":"A Esposito","year":"2013","unstructured":"Esposito A, Campana L, Palmisano A et al (2013) Magnetic resonance imaging at 7T reveals common events in age-related sarcopenia and in the homeostatic response to muscle sterile injury. PLoS ONE 8:e59308","journal-title":"PLoS ONE"},{"key":"1616_CR27","doi-asserted-by":"publisher","DOI":"10.1080\/2162402X.2018.1440929","volume":"7","author":"G Pacini","year":"2018","unstructured":"Pacini G, Cavalli G, Tomelleri A et al (2018) The fibrogenic chemokine CCL18 is associated with disease severity in Erdheim-Chester disease. Oncoimmunology 7:e1440929","journal-title":"Oncoimmunology"},{"key":"1616_CR28","doi-asserted-by":"publisher","first-page":"14151","DOI":"10.1038\/s41598-019-50478-5","volume":"9","author":"C Liang","year":"2019","unstructured":"Liang C, Wang K, Li Q et al (2019) Influence of the distribution of fibrosis within an area of myocardial infarction on wave propagation in ventricular tissue. Sci Rep 9:14151","journal-title":"Sci Rep"},{"key":"1616_CR29","doi-asserted-by":"publisher","first-page":"238","DOI":"10.3389\/fphys.2017.00238","volume":"8","author":"T Liu","year":"2017","unstructured":"Liu T, Song D, Dong J et al (2017) Current understanding of the pathophysiology of myocardial fibrosis and its quantitative assessment in heart failure. Front Physiol 8:238","journal-title":"Front Physiol"},{"key":"1616_CR30","doi-asserted-by":"publisher","first-page":"E114","DOI":"10.1002\/ajh.25055","volume":"93","author":"F Cohen-Aubart","year":"2018","unstructured":"Cohen-Aubart F, Emile J-F, Carrat F et al (2018) Phenotypes and survival in Erdheim-Chester disease: results from a 165-patient cohort. Am J Hematol 93:E114\u2013E117","journal-title":"Am J Hematol"},{"key":"1616_CR31","doi-asserted-by":"publisher","first-page":"1311","DOI":"10.1182\/blood.2019002766","volume":"135","author":"J Haroche","year":"2020","unstructured":"Haroche J, Cohen-Aubart F, Amoura Z (2020) Erdheim-Chester disease. Blood 135:1311\u20131318","journal-title":"Blood"},{"key":"1616_CR32","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1182\/bloodadvances.2016001784","volume":"1","author":"JI Estrada-Veras","year":"2017","unstructured":"Estrada-Veras JI, O\u2019Brien KJ, Boyd LC et al (2017) The clinical spectrum of Erdheim-Chester disease: an observational cohort study. Blood Adv 1:357\u2013366","journal-title":"Blood Adv"},{"key":"1616_CR33","doi-asserted-by":"publisher","first-page":"210","DOI":"10.3390\/jcm9010210","volume":"9","author":"A Schuster","year":"2020","unstructured":"Schuster A, Backhaus SJ, Stiermaier T et al (2020) Impact of right atrial physiology on heart failure and adverse events after myocardial infarction. J Clin Med 9:210","journal-title":"J Clin Med"},{"key":"1616_CR34","doi-asserted-by":"publisher","first-page":"1959","DOI":"10.1016\/j.jaccas.2020.08.014","volume":"2","author":"M Tao","year":"2020","unstructured":"Tao M, Palmer B, Tavakoli S, Trejo Bittar HE (2020) Erdheim-Chester Disease. JACC Case Rep 2:1959\u20131965","journal-title":"JACC Case Rep"},{"key":"1616_CR35","doi-asserted-by":"publisher","first-page":"682","DOI":"10.5858\/2004-128-682-MIIED","volume":"128","author":"AG Loeffler","year":"2004","unstructured":"Loeffler AG, Memoli VA (2004) Myocardial involvement in Erdheim-Chester disease. Arch Pathol Lab Med 128:682\u2013685","journal-title":"Arch Pathol Lab Med"}],"container-title":["La radiologia medica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11547-023-01616-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11547-023-01616-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11547-023-01616-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,20]],"date-time":"2023-04-20T13:53:02Z","timestamp":1681998782000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s11547-023-01616-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,22]]},"references-count":35,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2023,4]]}},"alternative-id":["1616"],"URL":"https:\/\/doi.org\/10.1007\/s11547-023-01616-7","relation":{},"ISSN":["1826-6983"],"issn-type":[{"value":"1826-6983","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,22]]},"assertion":[{"value":"7 January 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 February 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 March 2023","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Ethical approval was obtained from the San Raffaele Ethical Committee (PanImmuno Protocol).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"This is an observational study including human participants and it was performed in line with the principles of the Declaration of Helsinki.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical standards"}},{"value":"Written informed consent was obtained from all subjects (patients) in this study.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}]}}