{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T17:19:50Z","timestamp":1790961590873,"version":"4.1.0"},"reference-count":21,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2025,3,24]],"date-time":"2025-03-24T00:00:00Z","timestamp":1742774400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,28]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>OBJECTIVES<\/jats:title>\n                    <jats:p>The 2021 International Consensus for the congenital bicuspid aortic valve (BAV) condition recognizes 3 morphologies of BAV (fused, two-sinus and partial-fusion) and 3 types of aortopathy (ascending, root and extended). The clinical impact of BAV phenotyping on aortopathy evolution has not been evaluated so far. The aims were to assess: (i) prevalence of BAV phenotypes; (ii) frequency of BAV-related aortic valve dysfunction and aortopathy; and (iii) inter-phenotypic differences in aortopathy progression in a real-world population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>This was an observational cohort study on patients with BAV referred to our tertiary hospital between January 2018 and November 2022 to undergo a comprehensive transthoracic echocardiography. Baseline clinical, ultrasonographic and computed tomographic data were evaluated; even echocardiographic progression of aortic dilatation was assessed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>Three hundred and two patients were evaluated: 245 (81.1%) had fused, 41 (13.6%) two-sinus and 16 (5.3%) partial fusion BAV. Aortopathy was documented in 101 (33.6%) cases and it was prevalent among patients with the fused type. The prevalence of aortic valve dysfunction was instead similar among the 3 groups. Two hundred and twelve patients underwent invasive management of clinically relevant aortic valve or aortic disease. Non-operated fused type presented the highest progression rate of aortic dilatation, whilst, among the interventional subpopulation, a more pronounced evolution was observed in the two-sinus type, at a median follow-up of 2\u2009years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>Fused type represented the BAV phenotype with the highest frequency and the most significant association with aortopathy. In terms of aortopathic progression, the mid-term growth rate of the thoracic aorta was more significant in the non-interventional fused BAVs.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejcts\/ezaf109","type":"journal-article","created":{"date-parts":[[2025,3,23]],"date-time":"2025-03-23T07:37:10Z","timestamp":1742715430000},"source":"Crossref","is-referenced-by-count":6,"title":["Prevalence, clinical characterization, management and evolution of bicuspid aortic valve classified according to the 2021 International Consensus Statement in a tertiary care hospital"],"prefix":"10.1093","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-2348-826X","authenticated-orcid":false,"given":"Vincenzo","family":"Rizza","sequence":"first","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]},{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7972-0689","authenticated-orcid":false,"given":"Francesco","family":"Ancona","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giacomo","family":"Ingallina","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Stella","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Margonato","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Tavernese","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Belli","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Biondi","sequence":"additional","affiliation":[{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgio","family":"Fiore","sequence":"additional","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]},{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"Barki","sequence":"additional","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]},{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Damiano","family":"Cecchi","sequence":"additional","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Castiglioni","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9329-7437","authenticated-orcid":false,"given":"Michele","family":"De Bonis","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1065-8052","authenticated-orcid":false,"given":"Ottavio","family":"Alfieri","sequence":"additional","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]},{"name":"Cardiac Surgery Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Maisano","sequence":"additional","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]},{"name":"Cardiac Surgery Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4834-2187","authenticated-orcid":false,"given":"Eustachio","family":"Agricola","sequence":"additional","affiliation":[{"name":"Vita-Salute San Raffaele University , Milan,","place":["Italy"]},{"name":"Cardiovascular Imaging Unit, IRCCS San Raffaele Scientific Institute , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,3,24]]},"reference":[{"key":"2025041817063416500_ezaf109-B1","first-page":"72","article-title":"Bicuspid aortic valve","volume":"38","author":"Williams","year":"2006","journal-title":"J Insur Med"},{"key":"2025041817063416500_ezaf109-B2","doi-asserted-by":"crossref","first-page":"1920","DOI":"10.1056\/NEJMra1207059","article-title":"Aortic dilatation in patients with bicuspid aortic valve","volume":"370","author":"Verma","year":"2014","journal-title":"N Engl J Med"},{"key":"2025041817063416500_ezaf109-B3","doi-asserted-by":"crossref","first-page":"e383","DOI":"10.1016\/j.jtcvs.2021.06.019","article-title":"International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes","volume":"162","author":"Michelena","year":"2021","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2025041817063416500_ezaf109-B4","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/S0022-5223(19)34382-X","article-title":"The morphology of the normal aortic valve as compared with the aortic valve having two leaflets","volume":"98","author":"Angelini","year":"1989","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2025041817063416500_ezaf109-B5","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1016\/j.jtcvs.2007.01.039","article-title":"A classification system for the bicuspid aortic valve from 304 surgical specimens","volume":"133","author":"Sievers","year":"2007","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2025041817063416500_ezaf109-B6","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.jcmg.2012.11.007","article-title":"Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: comprehensive evaluation using MDCT and echocardiography","volume":"6","author":"Kang","year":"2013","journal-title":"JACC Cardiovasc Imaging"},{"key":"2025041817063416500_ezaf109-B7","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1016\/j.echo.2017.02.009","article-title":"Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography","volume":"30","author":"Baumgartner","year":"2017","journal-title":"J Am Soc Echocardiogr"},{"key":"2025041817063416500_ezaf109-B8","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.echo.2017.01.007","article-title":"Recommendations for noninvasive evaluation of native valvular regurgitation: a report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance","volume":"30","author":"Zoghbi","year":"2017","journal-title":"J Am Soc Echocardiogr"},{"key":"2025041817063416500_ezaf109-B9","doi-asserted-by":"crossref","first-page":"2873","DOI":"10.1093\/eurheartj\/ehu281","article-title":"2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC)","volume":"35","author":"Erbel","year":"2014","journal-title":"Eur Heart J"},{"key":"2025041817063416500_ezaf109-B10","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1093\/eurheartj\/ehab395","article-title":"2021 ESC\/EACTS Guidelines for the management of valvular heart disease","volume":"43","author":"Vahanian","year":"2022","journal-title":"Eur Heart J"},{"key":"2025041817063416500_ezaf109-B11","doi-asserted-by":"crossref","first-page":"e223","DOI":"10.1016\/j.jacc.2022.08.004","article-title":"2022 ACC\/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association\/American College of Cardiology Joint Committee on Clinical Practice Guidelines","volume":"80","author":"Isselbacher","year":"2022","journal-title":"J Am Coll Cardiol"},{"key":"2025041817063416500_ezaf109-B12","doi-asserted-by":"crossref","first-page":"100357","DOI":"10.1016\/j.ijcchd.2022.100357","article-title":"The genetic, molecular, and hemodynamic basis of bicuspid aortic valve aortopathy: a contemporary narrative review","volume":"8","author":"Burns","year":"2022","journal-title":"Int J Cardiol Congenital Heart Dis"},{"key":"2025041817063416500_ezaf109-B13","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1161\/CIRCIMAGING.112.973370","article-title":"Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta","volume":"5","author":"Barker","year":"2012","journal-title":"Circ Cardiovasc Imaging"},{"key":"2025041817063416500_ezaf109-B14","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1161\/CIRCULATIONAHA.113.003026","article-title":"Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy","volume":"129","author":"Mahadevia","year":"2014","journal-title":"Circulation"},{"key":"2025041817063416500_ezaf109-B15","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1186\/s12968-018-0451-1","article-title":"Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease","volume":"20","author":"Rodr\u00edguez-Palomares","year":"2018","journal-title":"J Cardiovasc Magn Reson"},{"key":"2025041817063416500_ezaf109-B16","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1097\/HCO.0000000000000601","article-title":"Utilizing wall shear stress as a clinical biomarker for bicuspid valve-associated aortopathy","volume":"34","author":"Hassanabad","year":"2019","journal-title":"Curr Opin Cardiol"},{"key":"2025041817063416500_ezaf109-B17","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.jcmg.2021.06.020","article-title":"Association of regional wall shear stress and progressive ascending aorta dilation in bicuspid aortic valve","volume":"15","author":"Soulat","year":"2022","journal-title":"JACC Cardiovasc Imaging"},{"key":"2025041817063416500_ezaf109-B18","doi-asserted-by":"crossref","first-page":"2707","DOI":"10.1161\/CIRCULATIONAHA.119.039693","article-title":"Partial aortic valve leaflet fusion is related to deleterious alteration of proximal aorta hemodynamics","volume":"139","author":"Guala","year":"2019","journal-title":"Circulation"},{"key":"2025041817063416500_ezaf109-B19","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.jtcvs.2015.04.020","article-title":"Greater asymmetric wall shear stress in Sievers\u2019 type 1\/LR compared with 0\/LAT bicuspid aortic valves after valve-sparing aortic root replacement","volume":"150","author":"Stephens","year":"2015","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2025041817063416500_ezaf109-B20","doi-asserted-by":"crossref","first-page":"629227","DOI":"10.3389\/fcvm.2021.629227","article-title":"Hemodynamic profiles before and after surgery in bicuspid aortic valve disease\u2014a systematic review of the literature","volume":"8","author":"Cave","year":"2021","journal-title":"Front Cardiovasc Med"},{"key":"2025041817063416500_ezaf109-B21","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1007\/s11886-022-01716-2","article-title":"Bicuspid aortic valves: an up-to-date review on genetics, natural history, and management","volume":"24","author":"Rashed","year":"2022","journal-title":"Curr Cardiol Rep"}],"container-title":["European Journal of Cardio-Thoracic Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ejcts\/advance-article-pdf\/doi\/10.1093\/ejcts\/ezaf109\/62593488\/ezaf109.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ejcts\/article-pdf\/67\/4\/ezaf109\/62593488\/ezaf109.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ejcts\/article-pdf\/67\/4\/ezaf109\/62593488\/ezaf109.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,18]],"date-time":"2025-04-18T17:06:48Z","timestamp":1744996008000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ejcts\/article\/doi\/10.1093\/ejcts\/ezaf109\/8092860"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,24]]},"references-count":21,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,3,28]]}},"URL":"https:\/\/doi.org\/10.1093\/ejcts\/ezaf109","relation":{},"ISSN":["1873-734X"],"issn-type":[{"value":"1873-734X","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,4]]},"published":{"date-parts":[[2025,3,24]]},"article-number":"ezaf109"}}