{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T20:51:39Z","timestamp":1789678299494,"version":"4.0.1"},"reference-count":142,"publisher":"Georg Thieme Verlag KG","issue":"01","license":[{"start":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T00:00:00Z","timestamp":1741824000000},"content-version":"vor","delay-in-days":12,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Liver Dis"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:p>Fontan-associated liver disease (FALD) occurs in all patients who have undergone Fontan palliation for functional single ventricle congenital heart defects. While liver fibrosis is universal in patients who have undergone Fontan palliation, FALD may lead to more serious consequences including portal hypertension, cirrhosis, and hepatocellular carcinoma. Scientific studies of the pathophysiology and clinical management of FALD have been limited to date by the heterogeneous nature of the disease, relatively small population of patients with Fontan physiology, and inaccuracy of noninvasive staging tests. As survival after the Fontan procedure improves, the population of adults with Fontan physiology is growing, leading to more severe extracardiac complications related to the Fontan circulation and growing demand for heart and liver transplantation. The accurate evaluation, staging, and management of FALD comprises a clinical challenge which requires expert multidisciplinary input.<\/jats:p>","DOI":"10.1055\/a-2556-4897","type":"journal-article","created":{"date-parts":[[2025,3,14]],"date-time":"2025-03-14T00:17:12Z","timestamp":1741911432000},"page":"114-128","source":"Crossref","is-referenced-by-count":8,"title":["Fontan-Associated Liver Disease"],"prefix":"10.1055","volume":"45","author":[{"given":"Moira B.","family":"Hilscher","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan N.","family":"Johnson","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2025,4,7]]},"reference":[{"issue":"01","key":"ref1","first-page":"39","article-title":"[\u201cCorrection\u201d of tricuspid atresia. 2 cases \u201ccorrected\u201d using a new surgical technic]","volume":"10","author":"F Fontan","year":"1971","journal-title":"Ann Chir Thorac Cardiovasc"},{"issue":"06","key":"ref2","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1136\/hrt.2004.051789","article-title":"The Fontan circulation","volume":"91","author":"M Gewillig","year":"2005","journal-title":"Heart"},{"issue":"05","key":"ref3","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1136\/hrt.66.5.372","article-title":"Doppler echocardiographic evaluation of pulmonary blood flow after the Fontan operation: the role of the lungs","volume":"66","author":"D J Penny","year":"1991","journal-title":"Br Heart J"},{"issue":"06","key":"ref4","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1055\/s-2000-8345","article-title":"Pathophysiological aspects after cavopulmonary anastomosis","volume":"48","author":"M Gewillig","year":"2000","journal-title":"Thorac Cardiovasc Surg"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.ahj.2024.10.008","article-title":"Fontan circulation and systemic disease - a retrospective cohort analysis over 35 years of follow-up","volume":"279","author":"G R Veldtman","year":"2025","journal-title":"Am Heart J"},{"issue":"05","key":"ref6","doi-asserted-by":"crossref","first-page":"3894","DOI":"10.1002\/ehf2.13294","article-title":"Reaching consensus for unified medical language in Fontan care","volume":"8","author":"T Alsaied","year":"2021","journal-title":"ESC Heart Fail"},{"issue":"04","key":"ref7","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1007\/s10741-020-09932-0","article-title":"Diagnosis and management of the adult patient with a failing Fontan circulation","volume":"25","author":"C R Broda","year":"2020","journal-title":"Heart Fail Rev"},{"issue":"06","key":"ref8","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1586\/erc.11.75","article-title":"The failing Fontan: etiology, diagnosis and management","volume":"9","author":"D J Goldberg","year":"2011","journal-title":"Expert Rev Cardiovasc Ther"},{"issue":"05","key":"ref9","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1016\/S0022-5223(98)70406-4","article-title":"Protein-losing enteropathy after the Fontan operation: an international multicenter study. 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