{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T22:09:15Z","timestamp":1788905355664,"version":"build-2803163510"},"reference-count":15,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,11,15]],"date-time":"2022-11-15T00:00:00Z","timestamp":1668470400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Fontan associated liver disease (FALD) potentially impacts Fontan patients undergoing heart transplant. This multi\u2010center study sought to identify pre\u2010transplant risk factors and characterize any post\u2010transplant liver recovery in those patients undergoing heart\u2010alone transplant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Review of Fontan patients at 12 pediatric institutions who underwent heart transplant between 2001\u20102019. Radiologists reviewed pre and post\u2010transplant liver imaging for fibrosis. Laboratory, pathology and endoscopy studies were reviewed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>156 patients underwent transplant due to decreased ventricular function (49%), protein losing enteropathy (31%) or plastic bronchitis (10%); median age at transplant was 13.6 years (interquartile range IQR 7.8, 17.2) with a median of 9.3 years (IQR 3.2, 13.4) between the Fontan operation and transplant. Few patients had pre\u2010transplant endoscopy (18%), and liver biopsy (19%). There were 31 deaths (20%). The median time from transplant to death was 0.5 years (95% Confidence Interval CI 0.0, 3.6). The five\u2010year survival was 73% (95% CI 64%, 83%). Deaths were related to cardiac causes in 68% (21\/31) and infection in 6 (19%). A pre\u2010transplant elevation in bilirubin was a predictor of death. Higher platelet levels were protective. Immediate post\u2010transplant elevations in creatinine, AST, ALT, and INR were predictive of death. Advanced liver fibrosis identified on ultrasound, computed tomography, or magnetic resonance imaging was not predictive of death. Liver imaging suggested some improvement in liver congestion post\u2010transplant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Elevated bilirubin, but not fibrosis on liver imaging, was associated with post\u2010heart transplant mortality in Fontan patients in this multicenter retrospective study. Additionally, heart transplant may alter the progression of FALD.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14435","type":"journal-article","created":{"date-parts":[[2022,11,16]],"date-time":"2022-11-16T06:09:43Z","timestamp":1668578983000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Fontan\u2010associated liver disease after heart transplant"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7679-5151","authenticated-orcid":false,"given":"Eric R.","family":"Griffiths","sequence":"first","affiliation":[{"name":"Pediatric Cardiothoracic Surgery University of Utah, Primary Children's Hospital  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8572-9874","authenticated-orcid":false,"given":"Linda M.","family":"Lambert","sequence":"additional","affiliation":[{"name":"Pediatric Cardiothoracic Surgery University of Utah, Primary Children's Hospital  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7353-6872","authenticated-orcid":false,"given":"Zhining","family":"Ou","sequence":"additional","affiliation":[{"name":"Division of Epidemiology, Department of Internal Medicine University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7748-1258","authenticated-orcid":false,"given":"Akraam","family":"Shaaban","sequence":"additional","affiliation":[{"name":"Department of Radiology University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2765-8414","authenticated-orcid":false,"given":"Maryam","family":"Rezvani","sequence":"additional","affiliation":[{"name":"Department of Radiology University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5460-9079","authenticated-orcid":false,"given":"Waldemar F.","family":"Carlo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Alabama Birmingham  Birmingham Alabama USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8659-736X","authenticated-orcid":false,"given":"Kurt R.","family":"Schumacher","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology, C.S. 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