{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T08:53:39Z","timestamp":1790758419741,"version":"4.1.0"},"reference-count":27,"publisher":"BMJ","issue":"1","license":[{"start":{"date-parts":[[2021,5,9]],"date-time":"2021-05-09T00:00:00Z","timestamp":1620518400000},"content-version":"unspecified","delay-in-days":8,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"NHMRC Project Grant","award":["1047923"],"award-info":[{"award-number":["1047923"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2021,2,22]]},"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Fontan-associated liver disease is accompanied by a hypercoagulable state. While hepatic dysfunction in Fontan patients is common, its relationship with haemostatic changes and clinical outcomes in this patient population remains unclear.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To correlate liver dysfunction and haemostatic profiles with clinical outcomes in the Fontan population.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Patients\/methods<\/jats:title>\n                  <jats:p>Patients were enrolled in a multicentre, cross-sectional study in Australia and New Zealand. Hepatic structure and function were assessed using serum-based calculations (Fibrotest and model for end-stage liver disease excluding international normalised ratio scores). Haemostatic profiles were assessed by Thrombin Generation. Platelet function was assessed via Platelet Factor 4 (PF4) and P-selectin (P-SEL). Clinical outcomes were obtained from the Australian and New Zealand Fontan Registry.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Seventy-three patients participated in the study (mean age 18.9\u00b18.5\u2009years with a mean of 13.5\u00b16.9\u2009years post-Fontan). The Endogenous Thrombin Potential (ETP) for patients who suffered thrombotic events (TE) (1366.4\u00b166.2\u2009nM\/min) was higher compared with patients with major bleeding events (1011.1\u00b1138.4\u2009nM\/min) (p=0.03). Except for a negative correlation between Fibrotest-score and PF4 (p=0.045), PF4 and P-SEL concentrations did not correlate with markers of hepatic dysfunction or structural abnormality.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Increased ETP is associated with TE during clinical follow-up after Fontan. This study reinforces that hepatic dysfunction may contribute to the derangement of coagulation factors, impacting the individual risk of haemostatic complications for the Fontan population.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/openhrt-2020-001460","type":"journal-article","created":{"date-parts":[[2021,5,10]],"date-time":"2021-05-10T13:10:41Z","timestamp":1620652241000},"page":"e001460","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":7,"title":["Cross-sectional assessment of haemostatic profile and hepatic dysfunction in Fontan patients"],"prefix":"10.1136","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5589-2806","authenticated-orcid":false,"given":"Joelle Julius Nicolaas","family":"Daems","sequence":"first","affiliation":[{"name":"Cardiology, University Medical Centre Amsterdam, Amsterdam, 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