{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T14:58:21Z","timestamp":1775487501263,"version":"3.50.1"},"reference-count":25,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,2,27]],"date-time":"2022-02-27T00:00:00Z","timestamp":1645920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>In Marfan syndrome, various cardiovascular pathologies, such as aortic dilatation and mitral valve pathologies, already occur in childhood and determine course of the disease. This study aimed to establish additional cardiovascular risk markers for severe Marfan phenotypes. We investigated tricuspid valve prolapse (TVP) and its predictive value for outcome of paediatric Marfan disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this retrospective, observational cohort study, we identified 130 paediatric Marfan patients (10.7\u00a0\u00b1\u00a04.8\u00a0years) with <jats:italic>FBN1<\/jats:italic> variants. We divided patients into two groups based on TVP presence and performed a cross\u2010sectional analysis to investigate the association of TVP with other cardiovascular, ocular and systemic pathologies, at first and last visit. A longitudinal analysis was performed with follow\u2010up data.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>At baseline, patients with TVP had higher incidence of aortic root dilatation (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.013), mitral valve prolapse (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.0001) and systemic manifestations (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.025) than patients without TVP. At follow\u2010up, previous presence of TVP predicted higher probability of aortic root dilatation (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.002), mitral valve prolapse (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.0001) and systemic manifestations (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.002).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This shows that TVP is linked to both cardiac and extracardiac Marfan manifestations and TVP is an important marker for a disease severity in these children. Therefore, TVP should be assessed routinely using echocardiography in paediatric Marfan patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.16307","type":"journal-article","created":{"date-parts":[[2022,2,23]],"date-time":"2022-02-23T08:18:52Z","timestamp":1645604332000},"page":"1261-1266","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Tricuspid valve prolapse as an early predictor for severe phenotype in children with Marfan syndrome"],"prefix":"10.1111","volume":"111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3978-1804","authenticated-orcid":false,"given":"Veronika C.","family":"Stark","sequence":"first","affiliation":[{"name":"Paediatric Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jakob","family":"Olfe","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jannis","family":"Pesch","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enver","family":"Tahir","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology and Nuclear Medicine University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julius M.","family":"Weinrich","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology and Nuclear Medicine University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Wiegand","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rainer","family":"Kozlik\u2010Feldmann","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yskert","family":"von Kodolitsch","sequence":"additional","affiliation":[{"name":"Department of Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas S.","family":"Mir","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology University Heart and Vascular Center University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,2,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010015\u20100369\u20108"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.76.1.41"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(05)67789\u20106"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCGEN.118.002185"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000223550.41849.e3"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1080\/14779072.2019.1704625"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2009.072785"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.12072"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijsu.2014.07.013"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.14503\/THIJ\u201013\u20103848"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735\u20101097(02)02161\u20107"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.21037\/cdt.2018.09.13"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/352337a0"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.15186"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2010.05.038"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.4414\/smw.2020.20189"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.5830\/CVJA\u20102010\u2010085"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1203\/PDR.0b013e3182097219"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002\u20108703(78)90083\u20102"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2013.123"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2008\u20100703"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.08.061"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246\u2010018\u20101880\u20101"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1136\/heart.87.5.470"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2012.82"}],"container-title":["Acta Paediatrica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.16307","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/apa.16307","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.16307","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,25]],"date-time":"2023-08-25T15:54:17Z","timestamp":1692978857000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apa.16307"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,27]]},"references-count":25,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2022,6]]}},"alternative-id":["10.1111\/apa.16307"],"URL":"https:\/\/doi.org\/10.1111\/apa.16307","archive":["Portico"],"relation":{},"ISSN":["0803-5253","1651-2227"],"issn-type":[{"value":"0803-5253","type":"print"},{"value":"1651-2227","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,27]]},"assertion":[{"value":"2022-01-06","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-21","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}