Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248

Article  CAS  PubMed  PubMed Central  Google Scholar 

d’Udekem Y, Iyengar AJ, Cochrane AD et al (2007) The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 116:I157–I164

Article  PubMed  Google Scholar 

Ohuchi H (2016) Adult patients with Fontan circulation: what we know and how to manage adults with Fontan circulation? J Cardiol 68:181–189

Article  PubMed  Google Scholar 

Dennis M, Zannino D, du Plessis K et al (2018) Clinical outcomes in adolescents and adults after the Fontan procedure. J Am Coll Cardiol 71:1009–1017

Article  PubMed  Google Scholar 

Alsaied T, Bokma JP, Engel ME et al (2017) Predicting long-term mortality after Fontan procedures: a risk score based on 6707 patients from 28 studies. Congenit Heart Dis 12:393–398

Article  PubMed  Google Scholar 

Stout KK, Daniels CJ, Aboulhosn JA et al (2019) 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 73:e81

Article  PubMed  Google Scholar 

Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M (1998) Protein-losing enteropathy after the Fontan operation: an international multicenter study. J Thorac Cardiovasc Surg 115:1063–1073

Article  CAS  PubMed  Google Scholar 

Budts W, Ravekes WJ, Danford DA, Kutty S (2020) Diastolic heart failure in patients with the Fontan circulation: a review. JAMA Cardiol 5:590

Article  PubMed  Google Scholar 

Rychik J, Atz AM, Celermajer DS et al (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 140:e234

Article  PubMed  Google Scholar 

Alsaied T, Bokma JP, Engel ME et al (2017) Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart 103:104–110

Article  PubMed  Google Scholar 

Gordon-Walker TT, Bove K, Veldtman G (2019) Fontan-associated liver disease: a review. J Cardiol 74(3):223–232

Article  PubMed  Google Scholar 

Pollak U et al (2018) Heparin-induced thrombocytopenia complicating children after the Fontan procedure: single-center experience and review of the literature. Congenit Heart Dis 13(1):16–25

Article  PubMed  Google Scholar 

Elder RW, McCabe NM, Hebson C et al (2013) Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol 168:3764–3769

Article  PubMed  PubMed Central  Google Scholar 

Narkewicz MR et al (2003) Hepatic dysfunction following the Fontan procedure. J Pediatr Gastroenterol Nutr 36(3):352–357

PubMed  Google Scholar 

Serai SD et al (2022) Liver and spleen volume and stiffness in patients post-Fontan procedure and patients with ARPKD compared to normal controls. Clin Imaging 89:147–154

Article  PubMed  Google Scholar 

Garcia-Peña J, Langham DR et al (2013) Fontan-associated liver disease: spectrum of US findings. Radiology 267(3):1–9. https://doi.org/10.1148/radiol.13121599

Article  Google Scholar 

Dillman JR, Trout AT, Alsaied T et al (2020) Imaging of Fontan-associated liver disease. Pediatr Radiol 50:1528–1541. https://doi.org/10.1007/s00247-020-04776-0

Article  PubMed  Google Scholar 

Gunsaulus M, Wang L, Haack L, Christopher A, Feingold B, Squires J, Horslen S, Hoskoppal A, Rose-Felker K, West S, Trucco S, Squires J, Olivieri L, Kreutzer J, Goldstein B, Alsaied T (2022) Cardiac MRI-derived inferior vena cava cross-sectional area correlates with measures of Fontan-associated liver disease. Pediatr Cardiol. https://doi.org/10.1007/s00246-022-03054-0

Article  PubMed  Google Scholar 

Jarasvaraparn C, Thoe J, Rodenbarger A, Masuoka H, Payne RM, Markham LW, Molleston JP (2024) Biomarkers of fibrosis and portal hypertension in Fontan-associated liver disease in children and adults. Dig Liver Dis. https://doi.org/10.1016/j.dld.2023.12.017

Article  PubMed  Google Scholar 

Lam CZ, Gulamhusein A, Wald RM (2021) Liver cirrhosis and hepatocellular carcinoma after the Fontan operation: reaching clarity in the face of uncertainty. Circulation 144(25):1977–1980. https://doi.org/10.1161/CIRCULATIONAHA.121.057431

Article  PubMed  Google Scholar 

Téllez L, Payancé A, Tjwa E, Del Cerro MJ, Idorn L, Ovroutski S, De Bruyne R, Verkade HJ, De Rita F, de Lange C, Angelini A, Paradis V, Rautou PE, García-Pagán JC (2023) EASL-ERN position paper on liver involvement in patients with Fontan-type circulation. J Hepatol 79(5):1270–1301. https://doi.org/10.1016/j.jhep.2023.07.013

Article  PubMed  Google Scholar 

Zafar F, Lubert AM, Trout AT, Katz DA, Palermo JJ, Opotowsky AR, Lorts A, Chin C, Powell AW, Villa CR, Anwar N, Morales DL, Dillman JR, Alsaied T (2022) Abdominal CT and MRI findings of portal hypertension in children and adults with Fontan circulation. Radiology 303(3):557–565. https://doi.org/10.1148/radiol.211037

Article  PubMed  Google Scholar 

Oechslin E (2015) Management of adults with cyanotic congenital heart disease. Heart 101(6):485–494

Article  PubMed  Google Scholar