{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T09:47:54Z","timestamp":1785318474628,"version":"3.55.0"},"reference-count":21,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2022,2,16]],"date-time":"2022-02-16T00:00:00Z","timestamp":1644969600000},"content-version":"vor","delay-in-days":15,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,2,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>At the most severe end of the spectrum of congenital heart disease are patients with an univentricular physiology. They comprise a heterogeneous group of congenital heart malformations that have the common characteristic that the cardiac morphology is not equipped for sustaining a biventricular circulation.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>Here, we present a case of an adult patient after Fontan palliation, illustrative of the complex clinical course and the broad spectrum of complications that can be encountered during follow-up, highlighting the need for a multidisciplinary approach in the clinical care for these patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>During the surgical Fontan procedure, the inferior vena cava is connected to the pulmonary circulation, after prior connection of the superior vena cava to the pulmonary arterial circulation. The resulting cavopulmonary connection, thus lacking a subpulmonic ventricle, provides non-pulsatile passive flow of oxygen-poor blood from the systemic venous circulation into the lungs, and the functional monoventricle pumps the oxygen-rich pulmonary venous return blood into the aorta. With an operative mortality of &amp;lt;5% and current 30-year survival rates up to 85%, the adult population of patients with a Fontan circulation is growing. This increase in survival is, however, inevitably accompanied by long-term complications affecting multiple organ systems, resulting in decline in cardiovascular performance.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>For optimal treatment, the evaluation in a multidisciplinary team is mandatory, using the specific expertise of the team members to timely detect and address late complications and to support quality of life.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytac067","type":"journal-article","created":{"date-parts":[[2022,2,16]],"date-time":"2022-02-16T14:53:44Z","timestamp":1645023224000},"source":"Crossref","is-referenced-by-count":6,"title":["Case report of the broad spectrum of late complications in an adult patient with univentricular physiology palliated by the Fontan circulation"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4893-6575","authenticated-orcid":false,"given":"Marieke","family":"Nederend","sequence":"first","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9312-2338","authenticated-orcid":false,"given":"Anastasia D","family":"Egorova","sequence":"additional","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6181-3377","authenticated-orcid":false,"given":"Hubert W","family":"Vliegen","sequence":"additional","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0153-5934","authenticated-orcid":false,"given":"Arno A W","family":"Roest","sequence":"additional","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Division of Paediatric Cardiology, Department of Paediatrics, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bastian N","family":"Ruijter","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Leiden University Medical Center, Leiden, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tijmen","family":"Korteweg","sequence":"additional","affiliation":[{"name":"Department of Radiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maarten K","family":"Ninaber","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7034-1017","authenticated-orcid":false,"given":"Katja","family":"Zeppenfeld","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3150-5007","authenticated-orcid":false,"given":"Mark G","family":"Hazekamp","sequence":"additional","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Department of Cardiothoracic Surgery, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1283-1122","authenticated-orcid":false,"given":"Philippine","family":"Ki\u00e8s","sequence":"additional","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9132-0418","authenticated-orcid":false,"given":"Monique R M","family":"Jongbloed","sequence":"additional","affiliation":[{"name":"CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands"},{"name":"Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"},{"name":"Department of Anatomy & Embryology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,2,16]]},"reference":[{"key":"2022022413101188800_ytac067-B1","doi-asserted-by":"crossref","first-page":"4153","DOI":"10.1093\/eurheartj\/ehaa701","article-title":"2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC)","volume":"41","author":"Baumgartner","year":"2020","journal-title":"Eur Heart J"},{"key":"2022022413101188800_ytac067-B2","doi-asserted-by":"crossref","first-page":"e234","DOI":"10.1161\/CIR.0000000000000696","article-title":"Evaluation and management of the child and adult with fontan circulation: a scientific statement from the American Heart 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Prevalence and predictors","volume":"32","author":"Magee","year":"1998","journal-title":"J Am Coll Cardiol"},{"key":"2022022413101188800_ytac067-B11","doi-asserted-by":"crossref","first-page":"E230","DOI":"10.1111\/chd.12276","article-title":"Embolization of veno-venous collaterals after the Fontan operation is associated with decreased survival","volume":"10","author":"Poterucha","year":"2015","journal-title":"Congenit Heart Dis"},{"key":"2022022413101188800_ytac067-B12","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/j.jjcc.2019.02.016","article-title":"Fontan-associated liver disease: a review","volume":"74","author":"Gordon-Walker","year":"2019","journal-title":"J Cardiol"},{"key":"2022022413101188800_ytac067-B13","doi-asserted-by":"crossref","first-page":"49","DOI":"10.2147\/JHC.S186239","article-title":"LI-RADS: a conceptual and historical review from its beginning to its recent integration into AASLD clinical practice guidance","volume":"6","author":"Elsayes","year":"2019","journal-title":"J Hepatocell Carcinoma"},{"key":"2022022413101188800_ytac067-B14","doi-asserted-by":"crossref","first-page":"2623","DOI":"10.1007\/s00261-017-1181-9","article-title":"Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma","volume":"42","author":"Wells","year":"2017","journal-title":"Abdom Radiol"},{"key":"2022022413101188800_ytac067-B15","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.jaccas.2019.05.031","article-title":"Fontan liver lesions: not always HCC","volume":"1","author":"Haeffele","year":"2019","journal-title":"JACC Case Rep"},{"key":"2022022413101188800_ytac067-B16","doi-asserted-by":"crossref","first-page":"2065","DOI":"10.1016\/j.jacc.2004.08.031","article-title":"Cardiac transplantation after the Fontan or Glenn procedure","volume":"44","author":"Jayakumar","year":"2004","journal-title":"J Am Coll Cardiol"},{"key":"2022022413101188800_ytac067-B17","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1007\/s12471-020-01505-y","article-title":"Waiting list mortality and the potential of donation after circulatory death heart transplantations in the Netherlands","volume":"29","author":"Roest","year":"2021","journal-title":"Neth Heart J"},{"key":"2022022413101188800_ytac067-B18","doi-asserted-by":"crossref","first-page":"e12892","DOI":"10.1111\/ctr.12892","article-title":"Single-center outcomes of combined heart and liver transplantation in the failing Fontan","volume":"31","author":"D'Souza","year":"2017","journal-title":"Clin Transplant"},{"key":"2022022413101188800_ytac067-B19","doi-asserted-by":"crossref","first-page":"e13329","DOI":"10.1111\/ctr.13329","article-title":"Heart and heart-liver transplantation in adults with failing Fontan physiology","volume":"32","author":"Reardon","year":"2018","journal-title":"Clin Transplant"},{"key":"2022022413101188800_ytac067-B20","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1053\/j.semtcvs.2020.09.005","article-title":"Mechanical support of the failing Fontan circulation","volume":"33","author":"Mascio","year":"2021","journal-title":"Semin Thorac Cardiovasc Surg"},{"key":"2022022413101188800_ytac067-B21","doi-asserted-by":"crossref","first-page":"802","DOI":"10.1161\/CIR.0000000000000353","article-title":"Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement from the American Heart Association","volume":"133","author":"Ross","year":"2016","journal-title":"Circulation"}],"container-title":["European Heart Journal - 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