{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T17:22:40Z","timestamp":1792430560797,"version":"4.5.0"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2024,2,2]],"date-time":"2024-02-02T00:00:00Z","timestamp":1706832000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,8,22]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35\u201348) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0\u201315.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4\u201310.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g\/L decrease, 95% confidence interval (CI): 1.04\u20131.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75\u201310.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23\u20137.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05\u201370.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/eurjpc\/zwae031","type":"journal-article","created":{"date-parts":[[2024,2,2]],"date-time":"2024-02-02T18:49:58Z","timestamp":1706899798000},"page":"1316-1323","source":"Crossref","is-referenced-by-count":28,"title":["Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life"],"prefix":"10.1093","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7899-463X","authenticated-orcid":false,"given":"Andrew","family":"Constantine","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7967-7417","authenticated-orcid":false,"given":"Paolo","family":"Ferrero","sequence":"additional","affiliation":[{"name":"ACHD Unit, IRCCS-Policlinico San Donato , San Donato Milanese, Milan , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Gribaudo","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0507-3060","authenticated-orcid":false,"given":"Panagiota","family":"Mitropoulou","sequence":"additional","affiliation":[{"name":"Congenital Cardiac Centre, Southampton General Hospital, University Hospital Southampton NHS Foundation Trust , Southampton , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaushiga","family":"Krishnathasan","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Costola","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myo T","family":"Lwin","sequence":"additional","affiliation":[{"name":"Congenital Cardiac Centre, Southampton General Hospital, University Hospital Southampton NHS Foundation Trust , Southampton , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Fitzsimmons","sequence":"additional","affiliation":[{"name":"Congenital Cardiac Centre, Southampton General Hospital, University Hospital Southampton NHS Foundation Trust , Southampton , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarita","family":"Brida","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"},{"name":"Department of Medical Rehabilitation, Medical Faculty University of Rijeka , Rijeka , Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8613-8139","authenticated-orcid":false,"given":"Claudia","family":"Montanaro","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksander","family":"Kempny","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ee Ling","family":"Heng","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7432-4815","authenticated-orcid":false,"given":"Massimo","family":"Chessa","sequence":"additional","affiliation":[{"name":"ACHD Unit, IRCCS-Policlinico San Donato , San Donato Milanese, Milan , Italy"},{"name":"UniSR - Vita Salute Sal Raffaele University , Milan , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1300-0504","authenticated-orcid":false,"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0107-7996","authenticated-orcid":false,"given":"Isma","family":"Rafiq","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust , Sydney Street, SW3 6NP London , UK"},{"name":"National Heart and Lung Institute, Imperial College London , Guy Scadding Building, Dovehouse Street, SW3 6LY London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,2,2]]},"reference":[{"key":"2024082123513165600_zwae031-B1","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1161\/CIRCULATIONAHA.105.592378","article-title":"Univentricular 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meta-analysis","volume":"104","author":"Schwartz","year":"2018","journal-title":"Heart"},{"key":"2024082123513165600_zwae031-B8","doi-asserted-by":"crossref","first-page":"e023035","DOI":"10.1161\/JAHA.121.023035","article-title":"Use of pulmonary arterial hypertension therapies in patients with a Fontan circulation: current practice across the United Kingdom","volume":"11","author":"Constantine","year":"2022","journal-title":"J Am Heart Assoc"},{"key":"2024082123513165600_zwae031-B9","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1161\/CIRCULATIONAHA.123.064400","article-title":"Survival from birth until young adulthood among individuals with congenital heart defects: CH STRONG","volume":"148","author":"Downing","year":"2023","journal-title":"Circulation"},{"key":"2024082123513165600_zwae031-B10","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1161\/CIRCULATIONAHA.107.738559","article-title":"Long-term survival, modes of death, and predictors of mortality in 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