{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:21:23Z","timestamp":1787509283129,"version":"build-2736575974"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2021,7,1]],"date-time":"2021-07-01T00:00:00Z","timestamp":1625097600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Stiftung KinderHerz"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,12,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>\u2002<\/jats:title>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBJECTIVES<\/jats:title>\n                    <jats:p>The Fontan operation aims at reducing cyanosis and cardiac volume overload in patients with complex univentricular heart malformations. However, persisting or reoccurring cyanosis is frequently observed. We sought to systematically determine the prevalence and clinical consequences of persisting and secondary cyanosis after Fontan operation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>A total of 331 Fontan patients, operated between 1984 and 2016 with a median postoperative follow-up of 7.9 (interquartile range 2.6\u201315.8) years, were studied retrospectively. Cyanosis was defined as transcutaneous oxygen saturation \u226493% at rest measured by pulse oximetry. Prevalence of cyanosis was analysed at 3 different time points (t1\u2009=\u2009post-Fontan operation, t2\u2009=\u2009post-Fontan cardiac catheterization, t3\u2009=\u2009last follow-up) and the association of cyanosis with mortality was examined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>Prevalence of cyanosis was 50% at t1 and 39% at t3. Fenestration was patent in 71% and 33% of all cyanotic patients at t1 and t3, respectively. In patients with clinical indication for catheterization (t2; n\u2009=\u2009178\/331), prevalence of cyanosis was 72%. At t2, patent fenestration (33%), veno-venous collaterals (24%) or both (32%) were present. Thirty-six (11%) patients died during follow-up. In a time-varying multivariable Cox regression analysis, cyanosis was the strongest predictor for late mortality (P\u2009&amp;lt;\u20090.001, hazard ratio 12.2, 95% confidence interval 3.7\u201340.5).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>Prevalence of cyanosis was considerable during long-term follow-up after Fontan operation and\u2014as a surrogate parameter for unfavourable Fontan haemodynamics\u2014is associated with increased late mortality. Accordingly, particular attention should be directed towards the persistence or reoccurrence of cyanosis during follow-up since it may indicate haemodynamic attrition and development of Fontan failure.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejcts\/ezab298","type":"journal-article","created":{"date-parts":[[2021,5,29]],"date-time":"2021-05-29T07:36:48Z","timestamp":1622273808000},"page":"54-61","source":"Crossref","is-referenced-by-count":21,"title":["Persisting and reoccurring cyanosis after Fontan operation is associated with increased late mortality"],"prefix":"10.1093","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4860-8010","authenticated-orcid":false,"given":"Marie","family":"Schafstedde","sequence":"first","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"},{"name":"Institute for Cardiovascular Computer-Assisted Medicine, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Berlin, Germany"},{"name":"Berlin Institute of Health, Berlin, Germany"},{"name":"DZHK (German Center for Cardiovascular Research), Partner Site Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1111-2521","authenticated-orcid":false,"given":"Sarah","family":"Nordmeyer","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"},{"name":"Institute for Cardiovascular Computer-Assisted Medicine, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2008-2483","authenticated-orcid":false,"given":"Anastasia","family":"Schleiger","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Nordmeyer","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7881-1557","authenticated-orcid":false,"given":"Felix","family":"Berger","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"},{"name":"DZHK (German Center for Cardiovascular Research), Partner Site Berlin, Germany"},{"name":"Department of Paediatric Cardiology, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4780-5271","authenticated-orcid":false,"given":"Peter","family":"Kramer","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanislav","family":"Ovroutski","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease\/Paediatric Cardiology, German Heart Center Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,7,1]]},"reference":[{"key":"2021122915015067100_ezab298-B1","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1136\/thx.26.3.240","article-title":"Surgical repair of tricuspid 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