{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:05:51Z","timestamp":1789016751644,"version":"build-2803163510"},"reference-count":32,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2025,7,22]],"date-time":"2025-07-22T00:00:00Z","timestamp":1753142400000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Pregnancy in women with a Fontan circulation carries increased risk. Given the relative evidence void, pregnancy counselling requires considerable nuance and experience.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>This study aimed to identify risk factors for maternal and foetal complications and to contrast risk estimates obtained from existing risk stratification tools, including the modified WHO, CARPREG II, and ZAHARA risk scores.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Pregnant women (&amp;gt;20 weeks of gestation) with a Fontan circulation were retrospectively included from 13 international centres. Univariate and multivariable analyses identified predictors of complications, and the performance of risk stratification tools was assessed. From 2006 to 2018, 84 women with Fontan physiology had 108 pregnancies and form the basis of this investigation. Maternal cardiovascular complications occurred in 32 (30%) of all pregnancies, including Fontan circulatory failure (17%), supraventricular tachycardia (7.4%), and thromboembolic events (3.7%). No maternal deaths occurred. Premature birth constituted 68% of neonatal complications, with a foetal and neonatal mortality of 13%. Multivariable analysis linked adverse maternal outcomes to pre-pregnancy oxygen saturation (adjusted OR, 0.77; 95%CI, 0.61\u20130.96; P = 0.02). None of the risk models showed good discriminative ability. The modified WHO classification was the only risk model significantly associated with adverse foetal outcomes.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Pregnancy in women with Fontan circulation poses a significant cardiovascular risk for the mother and a high burden of foetal and neonatal complications. The existing predictive risk stratification models do not discriminate hazard between well-functioning patients with Fontan from those with additional haemodynamic burden. This underscores the necessity for large-scale studies to refine risk stratification.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/eurjpc\/zwaf456","type":"journal-article","created":{"date-parts":[[2025,7,23]],"date-time":"2025-07-23T00:53:46Z","timestamp":1753232026000},"source":"Crossref","is-referenced-by-count":6,"title":["Refining current risk stratification guidelines for pregnant women with Fontan circulation: lessons from PROFAT registry"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4325-3521","authenticated-orcid":false,"given":"Magalie","family":"Ladouceur","sequence":"first","affiliation":[{"name":"Department of Cardiology, University Hospital of Geneva , Rue Gabrielle-Perret-Gentil 4, Geneva 1211 ,","place":["Switzerland"]},{"name":"Department of Cardiology, Adult Congenital Heart Disease Unit, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP , 20 rue Leblanc, 75015 Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Girnius","sequence":"additional","affiliation":[{"name":"Anesthesiology, University of Cincinnati College of Medicine , Cincinnati, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0437-1581","authenticated-orcid":false,"given":"Dominica","family":"Zentner","sequence":"additional","affiliation":[{"name":"Department of Cardiology and Department of Genomic Medicine, Royal Melbourne Hospital , Melbourne, Victoria ,","place":["Australia"]},{"name":"Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne , Melbourne, Victoria ,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4780-6936","authenticated-orcid":false,"given":"Anne Marie","family":"Valente","sequence":"additional","affiliation":[{"name":"Brigham and Women\u2019s Hospital, Department of Medicine, Division of Cardiovascular Disease, Department of Cardiology Harvard Medical School , 75 Francis Street, Boston, MA 02115 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine E","family":"Economy","sequence":"additional","affiliation":[{"name":"Brigham and Women\u2019s Hospital, Department of Obstetrics and Gynecology, Division Maternal and Fetal Medicine, Brigham and Women\u2019s Hospital , Boston, MA ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jolien","family":"Roos-Hesselink","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Erasmus MC, Rotterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carri","family":"Warshak","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology, University of Cincinnati College of Medicine , Cincinnati, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Partington","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine, Hospital of the University of Pennsylvania , Philadelphia, PA ,","place":["USA"]},{"name":"Division of Cardiology, The Children\u2019s Hospital of Philadelphia , Philadelphia, PA ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhiqian","family":"Gao","sequence":"additional","affiliation":[{"name":"The Heart Institute, Cincinnati Children\u2019s Hospital Medical Center , Cincinnati, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas J","family":"Ollberding","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine and Division of Biostatistics and Epidemiology, Cincinnati Children\u2019s Hospital Medical Center , Cincinnati, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Faust","sequence":"additional","affiliation":[{"name":"Heart Institute Research Core, Cincinnati Children\u2019s Hospital Medical Center , Cincinnati, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harald","family":"Kaemmerer","sequence":"additional","affiliation":[{"name":"Deutsches Herzzentrum M\u00fcnchen, Department of Congenital Heart Disease and Paediatric Cardiology, Germany , Munich ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teiji","family":"Akagi","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7312-6278","authenticated-orcid":false,"given":"Jasmine","family":"Grewal","sequence":"additional","affiliation":[{"name":"Cardiology, The University of British Columbia Faculty of Medicine , Vancouver, British Columbia ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa A","family":"Bradley","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease, Pennsylvania State University , Hershey, PA ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0975-7363","authenticated-orcid":false,"given":"Jonathan","family":"Buber","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiology, University of Washington School of Medicine , Seattle, Washington ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph S","family":"Palumbo","sequence":"additional","affiliation":[{"name":"Haematology, Cincinnati Children\u2019s Hospital Medical Center , Cincinnati, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niki","family":"Walker","sequence":"additional","affiliation":[{"name":"Scottish Adult Congenital Cardiac Service and University of Glasgow, Golden Jubilee National University Hospital , Glasgow ,","place":["United Kingdom"]},{"name":"Adult Congenital Heart Disease Center, Helen DeVos Children's Hospital, Corewell Health , Grand Rapids, MI ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0209-9505","authenticated-orcid":false,"given":"Jamil","family":"Aboulhosn","sequence":"additional","affiliation":[{"name":"Ahmanson\/UCLA ACHD Center, David Geffen School of Medicine at UCLA , Los Angeles,CA 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Wisconsin , Milwaukee, WI ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara J M","family":"Mulder","sequence":"additional","affiliation":[{"name":"Cardiology, Academic Medical Center , Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7084-8494","authenticated-orcid":false,"given":"Erwin","family":"Oechslin","sequence":"additional","affiliation":[{"name":"Toronto Adult Congenital Heart Disease Program, Peter Munk Cardiac Centre, University Health Network and University of Toronto , Toronto, Ontario ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9542-8821","authenticated-orcid":false,"given":"Helmut","family":"Baumgartner","sequence":"additional","affiliation":[{"name":"Department of Cardiology III: Adult Congenital and Valvular Heart Disease, University Hospital Muenster , Muenster, Albert 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