{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T17:13:55Z","timestamp":1790097235669,"version":"4.0.1"},"reference-count":24,"publisher":"Cambridge University Press (CUP)","issue":"9","license":[{"start":{"date-parts":[[2024,5,28]],"date-time":"2024-05-28T00:00:00Z","timestamp":1716854400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951124025241_as1\"><jats:title>Objectives:<\/jats:title><jats:p>We performed a single-centre retrospective study comparing the accuracy of non-invasive elastography with liver biopsy in accurate assessment of Fontan-associated liver disease.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124025241_as2\"><jats:title>Methods:<\/jats:title><jats:p>Fontan patients who underwent combined assessment with a percutaneous liver biopsy and non-invasive elastography between January 2015 and December 2023 at our Children\u2019s hospital were included. Liver biopsies were classified using the Congestive Hepatic Fibrosis Score as early Fontan-associated liver disease (scores 1, 2) and advanced Fontan-associated liver disease (score 3\/bridging fibrosis and score 4\/cirrhosis). Elastography values were categorised as advanced Fontan-associated liver disease for liver elasticity &gt;2.1 m\/s by ultrasound and liver stiffness &gt;5 KPa on magnetic resonance elastography.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124025241_as3\"><jats:title>Results:<\/jats:title><jats:p>We included 130 patients (116 children, 89%, mean age at biopsy: 14.6 years \u00b1 3.6) who underwent liver biopsy at a mean duration of 11.1 years (\u00b10.3) following Fontan surgery. Advanced Fontan-associated liver disease was noted in 41 (31.5%) patients with 13 (10%) showing frank cirrhosis. Pre-biopsy ultrasound showed advanced liver fibrosis in 18\/125 (14%), with low sensitivity (23%), high specificity (90%), and low accuracy (68%, <jats:italic>k<\/jats:italic> = 0.1) in diagnosing advanced Fontan-associated liver disease. Similarly, pre-biopsy magnetic resonance elastography showed advanced fibrosis in 23\/86 (27%) of patients, with low sensitivity (30%), fair specificity (75%), and low accuracy (63%, <jats:italic>k<\/jats:italic> = 0.1). Interestingly, advanced Fontan-associated liver disease was missed by ultrasound in 29% and by magnetic resonance elastography in 25% of patients. Advanced Fontan-associated liver disease was associated with lower platelet count (p = 0.02) and higher Gamma-glutamyl Transferase levels (p = 0.02).<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124025241_as4\"><jats:title>Conclusion:<\/jats:title><jats:p>Advanced hepatic fibrosis is common among paediatric Fontan patients. Non-invasive elastography may overestimate and underestimate the degree of liver fibrosis, and therefore, liver biopsy may be required for confirming disease severity.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951124025241","type":"journal-article","created":{"date-parts":[[2024,5,28]],"date-time":"2024-05-28T12:31:46Z","timestamp":1716899506000},"page":"1990-1996","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":5,"title":["Accuracy of elastography versus biopsy in assessing severity of liver fibrosis in young Fontan patients"],"prefix":"10.1017","volume":"34","author":[{"given":"Ozlem P.","family":"Bulut","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Smita S.","family":"Bailey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8733-8415","authenticated-orcid":false,"given":"Deepti P.","family":"Bhat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2024,5,28]]},"reference":[{"key":"S1047951124025241_ref17","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2015150619"},{"key":"S1047951124025241_ref22","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2021.100362"},{"key":"S1047951124025241_ref15","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.119.012529"},{"key":"S1047951124025241_ref20","doi-asserted-by":"publisher","DOI":"10.1148\/rg.2019190034"},{"key":"S1047951124025241_ref6","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.120.045597"},{"key":"S1047951124025241_ref18","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2020192437"},{"key":"S1047951124025241_ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2018.01.108"},{"key":"S1047951124025241_ref21","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2014.79"},{"key":"S1047951124025241_ref8","doi-asserted-by":"publisher","DOI":"10.1111\/chd.12650"},{"key":"S1047951124025241_ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2023.03.421"},{"key":"S1047951124025241_ref3","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.116.004809"},{"key":"S1047951124025241_ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2017.10.045"},{"key":"S1047951124025241_ref19","doi-asserted-by":"publisher","DOI":"10.1148\/rg.2016160042"},{"key":"S1047951124025241_ref10","doi-asserted-by":"publisher","DOI":"10.1016\/j.crad.2022.06.016"},{"key":"S1047951124025241_ref23","first-page":"1869","article-title":"Impact of sex, ethnicity, and body mass index on progression of fibrosis in Fontan-associated liver disease","volume":"70","author":"Emamaullee","year":"2019","journal-title":"Hepatology"},{"key":"S1047951124025241_ref2","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000696"},{"key":"S1047951124025241_ref1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jjcc.2019.02.016"},{"key":"S1047951124025241_ref13","doi-asserted-by":"publisher","DOI":"10.1111\/chd.12650"},{"key":"S1047951124025241_ref9","doi-asserted-by":"publisher","DOI":"10.1111\/chd.12770"},{"key":"S1047951124025241_ref24","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2015.04.020"},{"key":"S1047951124025241_ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2021.12.028"},{"key":"S1047951124025241_ref12","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-021-02707-w"},{"key":"S1047951124025241_ref16","doi-asserted-by":"publisher","DOI":"10.14740\/gr1557"},{"key":"S1047951124025241_ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2016.10.060"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951124025241","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,8]],"date-time":"2025-04-08T10:32:08Z","timestamp":1744108328000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951124025241\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,28]]},"references-count":24,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2024,9]]}},"alternative-id":["S1047951124025241"],"URL":"https:\/\/doi.org\/10.1017\/s1047951124025241","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"value":"1047-9511","type":"print"},{"value":"1467-1107","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,28]]},"assertion":[{"value":"\u00a9 Phoenix Children\u2019s Hospital, Arizona, United States, 2024. Published by Cambridge University Press","name":"copyright","label":"Copyright","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http:\/\/creativecommons.org\/licenses\/by\/4.0\/), which permits unrestricted re-use, distribution and reproduction, provided the original article is properly cited.","name":"license","label":"License","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}