{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T15:33:13Z","timestamp":1774452793478,"version":"3.50.1"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2021,1,5]],"date-time":"2021-01-05T00:00:00Z","timestamp":1609804800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,6,22]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Quadricuspid aortic valve (QAV) is a rare abnormality, which may cause aortic regurgitation (AR) requiring surgical intervention in some patients. The characteristics associated with aortic valve functional degeneration in patients with QAV are still unknown. The aim of this study is to describe QAV prevalence, characterize the disease by multimodality imaging, evaluate predictors of severe AR, and assess mid-term prognosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Retrospective search in imaging exams database of one tertiary centre, for patients diagnosed with QAV between January 2007 and September 2019. QAV was characterized by cardiac computed tomography, transthoracic\/transoesophageal echocardiography, and cardiac magnetic resonance. A total of 160\u2009004 exams were reviewed and eight patients with QAV were identified (50% men, mean age 53.5\u2009\u00b1\u200910.7\u2009years). The prevalence of QAV was 0.005%. During a median follow-up of 52\u2009months (interquartile range 16\u201388), there were no deaths. Seven patients (88%) had pure AR (three severe, one moderate, and three mild) and one patient (12%) had moderate AR and moderate aortic stenosis. Three patients (38%) with severe AR underwent valve surgery (two replacements and one repair). Analysis of predictors of severe AR was not statistically significant.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>QAV is a rare congenital cardiac defect, with a prevalence of 0.005% in our study. Its predominant functional abnormality was regurgitation and about one-third of the patients required aortic valve surgery. Multimodality imaging may play a pivotal role in assessing patients with QAV with significant valve dysfunction or associated congenital heart disease and improve their treatment strategy.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjci\/jeaa355","type":"journal-article","created":{"date-parts":[[2020,12,16]],"date-time":"2020-12-16T04:16:29Z","timestamp":1608092189000},"page":"765-776","source":"Crossref","is-referenced-by-count":11,"title":["Prevalence, multimodality imaging characterization, and mid-term prognosis of quadricuspid aortic valves: an analysis of eight cases, based on 160\u2009004 exams performed during 12\u2009years in a tertiary care hospital"],"prefix":"10.1093","volume":"22","author":[{"given":"Ana Mosalina","family":"Manuel","sequence":"first","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Ricardo","family":"Ladeiras-Lopes","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Jos\u00e9","family":"Ribeiro","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Nuno Dias","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Rita","family":"Faria","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"M\u00f3nica","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Wilson","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Ricardo","family":"Ferraz","sequence":"additional","affiliation":[{"name":"Cardiothoracic Surgery Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Fernando","family":"Gon\u00e7alves","sequence":"additional","affiliation":[{"name":"Cardiology Department, Tr\u00e1s-os-Montes e Alto Douro Hospital Centre, Vila Real, Portugal"}]},{"given":"Ana","family":"Batista","sequence":"additional","affiliation":[{"name":"Cardiology Department, Tr\u00e1s-os-Montes e Alto Douro Hospital Centre, Vila Real, Portugal"}]},{"given":"Carlos","family":"Gon\u00e7alves","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Gustavo","family":"Pires-Morais","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Alberto","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Francisco","family":"Sampaio","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"},{"name":"Cardiovascular Research & Development Unit, Department of Physiology and Cardiothoracic Surgery, Faculty of Medicine, University of Porto, Porto, Portugal"}]},{"given":"Madalena","family":"Teixeira","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Pedro","family":"Braga","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"}]},{"given":"Ricardo","family":"Fontes-Carvalho","sequence":"additional","affiliation":[{"name":"Cardiology Department, Vila Nova de Gaia\/Espinho Hospital Centre, Porto, Portugal"},{"name":"Cardiovascular Research & Development Unit, Department of Physiology and Cardiothoracic Surgery, Faculty of Medicine, University of Porto, Porto, Portugal"}]}],"member":"286","published-online":{"date-parts":[[2021,1,5]]},"reference":[{"key":"2021062219591852900_jeaa355-B1","author":"Balington","year":"1862"},{"key":"2021062219591852900_jeaa355-B2","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1016\/0002-9149(84)90546-0","article-title":"Diagnosis of incompetent quadricuspid aortic valve by two-dimensional echocardiography","volume":"53","author":"Herman","year":"1984","journal-title":"Am J Cardiol"},{"key":"2021062219591852900_jeaa355-B3","doi-asserted-by":"crossref","first-page":"941","DOI":"10.5761\/atcs.cr.13-00125","article-title":"Quadricuspid aortic valve: a report on a 10-year case series and literature review","volume":"20","author":"Hayakawa","year":"2014","journal-title":"Ann Thorac Cardiovasc Surg"},{"key":"2021062219591852900_jeaa355-B4","doi-asserted-by":"crossref","first-page":"177","DOI":"10.3390\/jcdd1030177","article-title":"Myths and realities relating to development of the arterial valves","volume":"1","author":"Anderson","year":"2014","journal-title":"J Cardiovasc Dev Dis"},{"key":"2021062219591852900_jeaa355-B5","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1002\/aja.1000710303","article-title":"The partitioning of the truncus and conus and the formation of the membranous portion of the interventricular septum in the human heart","volume":"71","author":"Kramer","year":"1942","journal-title":"Am J Anat"},{"key":"2021062219591852900_jeaa355-B6","doi-asserted-by":"crossref","first-page":"463","DOI":"10.3348\/kjr.2016.17.4.463","article-title":"Morphological and functional evaluation of quadricuspid aortic valves using cardiac computed tomography","volume":"17","author":"Song","year":"2016","journal-title":"Korean J Radiol"},{"key":"2021062219591852900_jeaa355-B7","first-page":"148","article-title":"Aggravating factors associated with the severity of aortic regurgitation and clinical characteristics of patients with quadricuspid aortic valve","volume":"24","author":"Cho","year":"2015","journal-title":"J Heart Valve Dis"},{"key":"2021062219591852900_jeaa355-B8","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1161\/CIRCULATIONAHA.115.017743","article-title":"Quadricuspid aortic valve: characteristics, associated structural cardiovascular abnormalities, and clinical outcomes","volume":"133","author":"Tsang","year":"2016","journal-title":"Circulation"},{"key":"2021062219591852900_jeaa355-B9","first-page":"454","article-title":"Quadricuspid aortic valve: a comprehensive review","volume":"31","author":"Yuan","year":"2016","journal-title":"Braz J Cardiovasc Surg"},{"key":"2021062219591852900_jeaa355-B10","doi-asserted-by":"crossref","first-page":"2739","DOI":"10.1093\/eurheartj\/ehx391","article-title":"2017 ESC\/EACTS guidelines for the management of valvular heart disease","volume":"38","author":"Baumgartner","year":"2017","journal-title":"Eur Heart J"},{"key":"2021062219591852900_jeaa355-B11","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1016\/0002-9149(73)90332-9","article-title":"Quadricuspid semilunar valve","volume":"31","author":"Hurwitz","year":"1973","journal-title":"Am J Cardiol"},{"key":"2021062219591852900_jeaa355-B12","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":"2021062219591852900_jeaa355-B13","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1016\/j.jcmg.2019.01.045","article-title":"Why and how to measure aortic valve calcification in patients with aortic stenosis","volume":"12","author":"Pawade","year":"2019","journal-title":"JACC Cardiovasc Imaging"},{"key":"2021062219591852900_jeaa355-B14","doi-asserted-by":"crossref","first-page":"1452","DOI":"10.1161\/CIRCULATIONAHA.111.083600","article-title":"Aortic regurgitation quantification using cardiovascular magnetic resonance: association with clinical outcome","volume":"126","author":"Myerson","year":"2012","journal-title":"Circulation"},{"key":"2021062219591852900_jeaa355-B15","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1016\/0002-9149(90)91446-D","article-title":"Incidence, description and functional assessment of isolated quadricuspid aortic valves","volume":"65","author":"Feldman","year":"1990","journal-title":"Am J Cardiol"},{"key":"2021062219591852900_jeaa355-B16","first-page":"134","article-title":"Congenital quadricuspid aortic valve: report of nine surgical cases","volume":"9","author":"Yotsumoto","year":"2003","journal-title":"Ann Thorac Cardiovasc Surg"},{"key":"2021062219591852900_jeaa355-B17","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1111\/j.1540-8175.2011.01477.x","article-title":"Quadricuspid aortic valve: a report of 12 cases and a review of the literature","volume":"28","author":"Jagannath","year":"2011","journal-title":"Echocardiography"},{"key":"2021062219591852900_jeaa355-B18","first-page":"792","article-title":"A previously undescribed type of quadricuspid aortic valve: type H","volume":"19","author":"Vali","year":"2010","journal-title":"J Heart Valve Dis"},{"key":"2021062219591852900_jeaa355-B19","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1007\/BF02913511","article-title":"Quadricuspid aortic valve associated with aortic stenosis and regurgitation","volume":"49","author":"Nakamura","year":"2001","journal-title":"Jpn J Thorac Cardiovasc Surg"},{"key":"2021062219591852900_jeaa355-B20","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1111\/echo.14278","article-title":"Long-term outcomes in 3 cases of quadricuspid aortic valve, using a new classification system: a case series and literature review","volume":"36","author":"Tsugu","year":"2019","journal-title":"Echocardiography"},{"key":"2021062219591852900_jeaa355-B21","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1093\/ehjci\/jex344","article-title":"Echocardiographic criteria to detect unicuspid aortic valve morphology","volume":"20","author":"Ewen","year":"2019","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"2021062219591852900_jeaa355-B22","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1016\/j.echo.2016.08.017","article-title":"The significance of the interleaflet triangles in determining the morphology of congenitally abnormal aortic valves: implications for noninvasive imaging and surgical management","volume":"29","author":"Tretter","year":"2016","journal-title":"J Am Soc Echocardiogr"},{"key":"2021062219591852900_jeaa355-B23","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.jtcvs.2015.03.019","article-title":"Outcomes after repair or replacement of dysfunctional quadricuspid aortic valve","volume":"150","author":"Idrees","year":"2015","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2021062219591852900_jeaa355-B24","first-page":"534","article-title":"The quadricuspid aortic valve: a comprehensive review","volume":"13","author":"Tutarel","year":"2004","journal-title":"J Heart Valve Dis"},{"key":"2021062219591852900_jeaa355-B25","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1007\/s004150050414","article-title":"Neurological involvement and quadricuspid aortic valve in a patient with Ehlers-Danlos syndrome","volume":"246","author":"Dotti","year":"1999","journal-title":"J Neurol"},{"key":"2021062219591852900_jeaa355-B26","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1016\/j.repc.2011.09.007","article-title":"Quadricuspid aortic valve\u201310-year case series and literature review","volume":"30","author":"Gouveia","year":"2011","journal-title":"Rev Port Cardiol"},{"key":"2021062219591852900_jeaa355-B27","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1186\/1749-8090-9-29","article-title":"Mid-term results in patients having tricuspidization of the quadricuspid aortic valve","volume":"9","author":"Song","year":"2014","journal-title":"J Cardiothorac Surg"},{"key":"2021062219591852900_jeaa355-B28","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1002\/clc.4950251203","article-title":"Quadricuspid aortic valves","volume":"25","author":"Timperley","year":"2002","journal-title":"Clin Cardiol"},{"key":"2021062219591852900_jeaa355-B29","first-page":"2779","article-title":"Congenital quadricuspid aortic valve: analysis of 11 surgical cases","volume":"124","author":"Tang","year":"2011","journal-title":"Chin Med J (Engl)"}],"container-title":["European Heart Journal - Cardiovascular Imaging"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/ehjcimaging\/article-pdf\/22\/7\/765\/38813524\/jeaa355.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/ehjcimaging\/article-pdf\/22\/7\/765\/38813524\/jeaa355.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,22]],"date-time":"2021-06-22T20:00:19Z","timestamp":1624392019000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcimaging\/article\/22\/7\/765\/6064831"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,5]]},"references-count":29,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2021,1,5]]},"published-print":{"date-parts":[[2021,6,22]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjci\/jeaa355","relation":{},"ISSN":["2047-2404","2047-2412"],"issn-type":[{"value":"2047-2404","type":"print"},{"value":"2047-2412","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,7,1]]},"published":{"date-parts":[[2021,1,5]]}}}