Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019;140:e234–e284
DiPaola FW, Schumacher KR, Goldberg CS, Friedland-Little J, Parameswaran A, Dillman JR. Effect of Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol. 2017;27:2434–2442
Emamaullee J, Khan S, Weaver C, Goldbeck C, Yanni G, Kohli R, et al. Non-invasive biomarkers of Fontan-associated liver disease. JHEP Reports. 2021;3:100362
PubMed PubMed Central Google Scholar
Jarasvaraparn C, Thoe J, Rodenbarger A, Masuoka H, Payne RM, Markham LW, et al. Biomarkers of fibrosis and portal hypertension in Fontan-associated liver disease in children and adults. Digestive and Liver Disease. 2024;56:1335–1342
Munsterman ID, Duijnhouwer AL, Kendall TJ, Bronkhorst CM, Ronot M, van Wettere M, et al. The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort. Eur Heart J. 2019;40:1057–1068
Witters P, Freson K, Verslype C, Peerlinck K, Hoylaerts M, Nevens F, et al. Blood platelet number and function in chronic liver disease and cirrhosis. Aliment Pharmacol Ther. 2008;27:1017–1029
Afdhal N, McHutchison J, Brown R, Jacobson I, Manns M, Poordad F, et al. Thrombocytopenia associated with chronic liver disease. J Hepatol. 2008;48:1000–1007
Peng Y, Qi X, Guo X. Child-Pugh versus MELD score for the assessment of prognosis in liver cirrhosis: a systematic review and meta-analysis of observational studies. Medicine. 2016;95:e2877
PubMed PubMed Central Google Scholar
Heuman DM, Mihas AA, Habib A, Gilles HS, Stravitz RT, Sanyal AJ, et al. MELD-XI: a rational approach to “sickest first” liver transplantation in cirrhotic patients requiring anticoagulant therapy. Liver Transpl. 2007;13:30–37
Lin ZH, Xin YN, Dong QJ, Wang Q, Jiang XJ, Zhan SH, et al. Performance of the aspartate aminotransferase-to-platelet ratio index for the staging of hepatitis C-related fibrosis: an updated meta-analysis. Hepatology. 2011;53:726–736
Vallet-Pichard A, Mallet V, Nalpas B, Verkarre V, Nalpas A, Dhalluin-Venier V, et al. FIB-4: an inexpensive and accurate marker of fibrosis in HCV infection. comparison with liver biopsy and fibrotest. Hepatology. 2007;46:32–36
Elder RW, McCabe NM, Hebson C, Veledar E, Romero R, Ford RM, et al. Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol. 2013;168:3764–3769
PubMed PubMed Central Google Scholar
Dai D-F, Swanson PE, Krieger EV, Liou IW, Carithers RL, Yeh MM. Congestive hepatic fibrosis score: a novel histologic assessment of clinical severity. Mod Pathol. 2014;27:1552–1558
Schleiger A, Salzmann M, Kramer P, Danne F, Schubert S, Bassir C, et al. Severity of fontan-associated liver disease correlates with fontan hemodynamics. Pediatr Cardiol. 2020;41:736–746
PubMed PubMed Central Google Scholar
Pundi K, Pundi KN, Kamath PS, Cetta F, Li Z, Poterucha JT, et al. Liver disease in patients after the Fontan operation. Am J Cardiol. 2016;117:456–460
Lindsay I, Johnson J, Everitt MD, Hoffman J, Yetman AT. Impact of liver disease after the Fontan operation. Am J Cardiol. 2015;115:249–252
Ofei SY, Gariepy C, Hanje J, Sisk T, Daniels CJ, Zaidi AN. Liver fibrosis in adults with Fontan palliation: do common screening studies predict disease severity? Int J Cardiol. 2015;181:174–175
Yoneda M, Fujii H, Sumida Y, Hyogo H, Itoh Y, Ono M, et al. Platelet count for predicting fibrosis in nonalcoholic fatty liver disease. J Gastroenterol. 2011;46:1300–1306
Murali AR, Attar BM, Katz A, Kotwal V, Clarke PM. Utility of platelet count for predicting cirrhosis in alcoholic liver disease: model for identifying cirrhosis in a US population. J Gen Intern Med. 2015;30:1112–1117
PubMed PubMed Central Google Scholar
Tripodi A, Mannucci PM. Abnormalities of hemostasis in chronic liver disease: reappraisal of their clinical significance and need for clinical and laboratory research. J Hepatol. 2007;46:727–733
Pasquet J-m, Gross BS, Gratacap M-P, Quek L, Pasquet S, Payrastre B, et al. Thrombopoietin potentiates collagen receptor signaling in platelets through a phosphatidylinositol 3-kinase–dependent pathway. Blood, J Am Soc Hematol. 2000;95:3429–3434
Mitchell O, Feldman DM, Diakow M, Sigal SH (2016) The pathophysiology of thrombocytopenia in chronic liver disease. Hepatic medicine: evidence and research, 39–50.
Giannini E, Borro P, Botta F, Fumagalli A, Malfatti F, Podestà E, et al. Serum thrombopoietin levels are linked to liver function in untreated patients with hepatitis C virus-related chronic hepatitis. J Hepatol. 2002;37:572–577
Weksler B. the pathophysiology of thrombocytopenia in hepatitis C virus infection and chronic liver disease. Aliment Pharmacol Ther. 2007;26:13–19
Gould M, Gunsaulus M, Feingold B, Goldstein B, Hoskoppal A, Kreutzer J, et al. Thrombocytopenia is associated with higher fontan pressure and increased morbidity in patients with fontan circulation. Pediatr Cardiol. 2024. https://doi.org/10.1007/s00246-024-03567-w
Agnoletti G, Ferraro G, Bordese R, Marini D, Gala S, Bergamasco L, et al. Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy? Int J Cardiol. 2016;209:60–65
Evans WN, Acherman RJ, Ciccolo ML, Carrillo SA, Galindo A, Rothman A, et al. MELD-XI scores correlate with post-Fontan hepatic biopsy fibrosis scores. Pediatr Cardiol. 2016;37:1274–1277
Guerrero-Chalela CE, Therrien J, Grossman Y, Guo L, Liu A, Marelli A. Severe fontan associated liver disease and its association with mortality. J Am Heart Assoc. 2023;12:e024034
PubMed PubMed Central Google Scholar
Simpson KE, Esmaeeli A, Khanna G, White F, Turnmelle Y, Eghtesady P, et al. Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function. J Heart Lung Transplant. 2014;33:170–177
Lewis MJ, Reardon LC, Aboulhosn J, Haeffele C, Chen S, Kim Y, et al. Clinical outcomes of adult fontan-associated liver disease and combined heart-liver transplantation. J Am Coll Cardiol. 2023;81:2149–2160
Comments (0)