{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T11:35:15Z","timestamp":1789644915786,"version":"4.0.0"},"reference-count":162,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T00:00:00Z","timestamp":1779148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T00:00:00Z","timestamp":1779148800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Curr Cardiol Rep"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose of Review<\/jats:title>\n                    <jats:p>This manuscript reviews the clinical spectrum and management of Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE), examining how chronic venous hypertension leads to multisystem injury.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Recent Findings<\/jats:title>\n                    <jats:p>Liver fibrosis is now recognized as an early and nearly universal complication after the Fontan procedure, with cirrhosis affecting approximately 43% of patients by 30\u00a0years post-operation. Although post-2001 survival exceeds 90%, standard biomarkers and imaging frequently underestimate disease severity, as liver stiffness measurements are confounded by hepatic congestion. Liver biopsy remains the gold standard for staging.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Summary<\/jats:title>\n                    <jats:p>FALD is an inevitable consequence of Fontan physiology, characterized by sinusoidal congestion and progressive fibrosis, while PLE results from multifactorial gastrointestinal protein loss. The key clinical implication is the importance of a multidisciplinary approach, particularly when considering transition from isolated heart transplantation to combined heart\u2013liver transplantation in advanced disease. Future research should prioritize standardized staging systems and targeted therapies to reduce lymphatic dysfunction and fibrosis progression.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11886-026-02378-0","type":"journal-article","created":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T09:14:04Z","timestamp":1779182044000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and Protein-Losing Enteropathy"],"prefix":"10.1007","volume":"28","author":[{"given":"Chaowapong","family":"Jarasvaraparn","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary R.","family":"Schooler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iv\u00e1n A.","family":"Gonz\u00e1lez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salil","family":"Ginde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alisha","family":"Mavis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,19]]},"reference":[{"key":"2378_CR1","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1136\/thx.26.3.240","volume":"26","author":"F Fontan","year":"1971","unstructured":"Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240\u20138.","journal-title":"Thorax"},{"key":"2378_CR2","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1016\/S0022-5223(19)40598-9","volume":"66","author":"G Kreutzer","year":"1973","unstructured":"Kreutzer G, Gal\u00edndez E, Bono H, de Palma C, Laura JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1973;66:613\u201321.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR3","doi-asserted-by":"publisher","first-page":"1700","DOI":"10.1016\/j.jacc.2015.07.065","volume":"66","author":"KN Pundi","year":"2015","unstructured":"Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, et al. 40-year follow-up after the Fontan operation: long-term outcomes of 1,052 patients. J Am Coll Cardiol. 2015;66:1700\u201310.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR4","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1007\/s12325-021-02002-3","volume":"39","author":"L Plappert","year":"2022","unstructured":"Plappert L, Edwards S, Senatore A, De Martini A. The epidemiology of persons living with Fontan in 2020 and projections for 2030: development of an epidemiology model providing multinational estimates. Adv Ther. 2022;39:1004\u201315.","journal-title":"Adv Ther"},{"key":"2378_CR5","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1016\/j.ijcard.2016.05.035","volume":"219","author":"C Schilling","year":"2016","unstructured":"Schilling C, Dalziel K, Nunn R, Du Plessis K, Shi WY, Celermajer D, et al. The Fontan epidemic: population projections from the Australia and New Zealand Fontan Registry. Int J Cardiol. 2016;219:14\u20139.","journal-title":"Int J Cardiol"},{"key":"2378_CR6","doi-asserted-by":"publisher","first-page":"1311","DOI":"10.1016\/j.ijcard.2012.03.182","volume":"167","author":"L Idorn","year":"2013","unstructured":"Idorn L, Olsen M, Jensen AS, Juul K, Reimers JI, S\u00f8rensen K, et al. Univentricular hearts in Denmark 1977 to 2009: incidence and survival. Int J Cardiol. 2013;167:1311\u20136.","journal-title":"Int J Cardiol"},{"key":"2378_CR7","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1055\/s-2000-8345","volume":"48","author":"M Gewillig","year":"2000","unstructured":"Gewillig M, Kalis N. Pathophysiological aspects after cavopulmonary anastomosis. Thorac Cardiovasc Surg. 2000;48:336\u201341.","journal-title":"Thorac Cardiovasc Surg"},{"key":"2378_CR8","doi-asserted-by":"publisher","first-page":"682","DOI":"10.1016\/S0022-5223(19)35174-8","volume":"96","author":"MR de Leval","year":"1988","unstructured":"de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. experimental studies and early clinical experience. J Thorac Cardiovasc Surg. 1988;96:682\u201395.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR9","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1067\/mtc.2001.111422","volume":"121","author":"C Stamm","year":"2001","unstructured":"Stamm C, Friehs I, Mayer JE Jr, Zurakowski D, Triedman JK, Moran AM, et al. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001;121:28\u201341.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR10","doi-asserted-by":"publisher","first-page":"1219","DOI":"10.1067\/mtc.2001.116947","volume":"122","author":"A Azakie","year":"2001","unstructured":"Azakie A, McCrindle BW, Van Arsdell G, Benson LN, Coles J, Hamilton R, et al. Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes. J Thorac Cardiovasc Surg. 2001;122:1219\u201328.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR11","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1016\/j.jjcc.2019.02.016","volume":"74","author":"TT Gordon-Walker","year":"2019","unstructured":"Gordon-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: a review. J Cardiol. 2019;74:223\u201332.","journal-title":"J Cardiol"},{"key":"2378_CR12","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1177\/2150135121989974","volume":"12","author":"J Rychik","year":"2021","unstructured":"Rychik J, Goldmuntz E. Invited commentary: the hunt for mechanistic origins of liver fibrosis in the Fontan circulation. World J Pediatr Congenit Heart Surg. 2021;12:173\u20135.","journal-title":"World J Pediatr Congenit Heart Surg"},{"key":"2378_CR13","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1016\/j.ijcard.2020.05.064","volume":"317","author":"E Abbasi Bavil","year":"2020","unstructured":"Abbasi Bavil E, Yang HK, Doyle MG, Kim TK, Karur G, Bhagra C, et al. Cardiovascular and abdominal flow alterations in adults with morphologic evidence of liver disease post Fontan palliation. Int J Cardiol. 2020;317:63\u20139.","journal-title":"Int J Cardiol"},{"key":"2378_CR14","doi-asserted-by":"publisher","first-page":"e234","DOI":"10.1161\/CIR.0000000000000696","volume":"140","author":"J Rychik","year":"2019","unstructured":"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\u201384.","journal-title":"Circulation"},{"key":"2378_CR15","doi-asserted-by":"publisher","first-page":"2298","DOI":"10.1016\/j.athoracsur.2015.06.071","volume":"100","author":"MC Schwartz","year":"2015","unstructured":"Schwartz MC, Glatz AC, Daniels K, Goldberg DJ, Rand E, Epelman MS, et al. Hepatic abnormalities are present before and early after the Fontan operation. Ann Thorac Surg. 2015;100:2298\u2013304.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR16","doi-asserted-by":"publisher","first-page":"904","DOI":"10.1016\/j.jtcvs.2011.08.038","volume":"143","author":"MC Schwartz","year":"2012","unstructured":"Schwartz MC, Sullivan L, Cohen MS, Russo P, John AS, Guo R, et al. Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy study. J Thorac Cardiovasc Surg. 2012;143:904\u20139.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR17","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1016\/j.jtcvs.2012.09.005","volume":"146","author":"JA Johnson","year":"2013","unstructured":"Johnson JA, Cetta F, Graham RP, Smyrk TC, Driscoll DJ, Phillips SD, et al. Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis. J Thorac Cardiovasc Surg. 2013;146:140\u20135.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR18","doi-asserted-by":"publisher","first-page":"648","DOI":"10.1002\/hep.27387","volume":"61","author":"DA Simonetto","year":"2015","unstructured":"Simonetto DA, Yang HY, Yin M, de Assuncao TM, Kwon JH, Hilscher M, et al. Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology. 2015;61:648\u201359.","journal-title":"Hepatology"},{"key":"2378_CR19","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1055\/a-2556-4897","volume":"45","author":"MB Hilscher","year":"2025","unstructured":"Hilscher MB, Johnson JN. Fontan-associated liver disease. Semin Liver Dis. 2025;45:114\u201328.","journal-title":"Semin Liver Dis"},{"key":"2378_CR20","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1053\/j.gastro.2019.03.013","volume":"157","author":"MB Hilscher","year":"2019","unstructured":"Hilscher MB, Sehrawat T, Arab JP, Zeng Z, Gao J, Liu M, et al. Mechanical stretch increases expression of CXCL1 in liver sinusoidal endothelial cells to recruit neutrophils, generate sinusoidal microthombi, and promote portal hypertension. Gastroenterology. 2019;157:193-209.e199.","journal-title":"Gastroenterology"},{"key":"2378_CR21","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1016\/j.cld.2018.12.004","volume":"23","author":"MB Hilscher","year":"2019","unstructured":"Hilscher MB, Kamath PS. The liver in circulatory disturbances. Clin Liver Dis. 2019;23:209\u201320.","journal-title":"Clin Liver Dis"},{"key":"2378_CR22","doi-asserted-by":"publisher","DOI":"10.1136\/openhrt-2020-001434","volume":"7","author":"T Alsaied","year":"2020","unstructured":"Alsaied T, Moore RA, Lang SM, Truong V, Lubert AM, Veldtman GR, et al. Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation. Open Heart. 2020;7:7.","journal-title":"Open Heart"},{"key":"2378_CR23","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1016\/j.ijcard.2020.01.012","volume":"305","author":"A Saraf","year":"2020","unstructured":"Saraf A, De Staercke C, Everitt I, Haouzi A, Ko YA, Jennings S, et al. Biomarker profile in stable Fontan patients. Int J Cardiol. 2020;305:56\u201362.","journal-title":"Int J Cardiol"},{"key":"2378_CR24","volume":"19","author":"A Angelotti","year":"2025","unstructured":"Angelotti A, Dhesi M, Bansal SS, Bradley EA. Novel immunologic mechanisms for Fontan-associated liver disease. Int J Cardiol Congenit Heart Dis. 2025;19:100554.","journal-title":"Int J Cardiol Congenit Heart Dis"},{"key":"2378_CR25","doi-asserted-by":"publisher","first-page":"638","DOI":"10.1111\/liv.14375","volume":"40","author":"E Rodr\u00edguez de Santiago","year":"2020","unstructured":"Rodr\u00edguez de Santiago E, T\u00e9llez L, Garrido-Lestache Rodr\u00edguez-Monte E, Garrido-G\u00f3mez E, Aguilera-Castro L, \u00c1lvarez-Fuente M, et al. Fontan protein-losing enteropathy is associated with advanced liver disease and a proinflammatory intestinal and systemic state. Liver Int. 2020;40:638\u201345.","journal-title":"Liver Int"},{"key":"2378_CR26","doi-asserted-by":"publisher","first-page":"99","DOI":"10.3350\/cmh.2013.19.2.99","volume":"19","author":"C Chung","year":"2013","unstructured":"Chung C, Iwakiri Y. The lymphatic vascular system in liver diseases: its role in ascites formation. Clin Mol Hepatol. 2013;19:99\u2013104.","journal-title":"Clin Mol Hepatol"},{"key":"2378_CR27","first-page":"114","volume":"49","author":"H James","year":"2016","unstructured":"James H, Witte MH, Bernas M, Barber B. Proposal for prevention or alleviation of protein\/lymph-losing enteropathy (PLE\/LLE) after Fontan circulation treatment of univentricular hearts: restoration of lymph balance with a \u201clymphatic right-to-left shunt.\u201d Lymphology. 2016;49:114\u201327.","journal-title":"Lymphology"},{"key":"2378_CR28","doi-asserted-by":"publisher","first-page":"1561","DOI":"10.1016\/j.jacc.2007.07.016","volume":"50","author":"A Sandek","year":"2007","unstructured":"Sandek A, Bauditz J, Swidsinski A, Buhner S, Weber-Eibel J, von Haehling S, et al. Altered intestinal function in patients with chronic heart failure. J Am Coll Cardiol. 2007;50:1561\u20139.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR29","doi-asserted-by":"publisher","first-page":"1307","DOI":"10.1016\/j.amjcard.2007.05.059","volume":"100","author":"A Wang","year":"2007","unstructured":"Wang A, Book WM, McConnell M, Lyle T, Rodby K, Mahle WT. Prevalence of hepatitis C infection in adult patients who underwent congenital heart surgery prior to screening in 1992. Am J Cardiol. 2007;100:1307\u20139.","journal-title":"Am J Cardiol"},{"key":"2378_CR30","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1016\/j.amjcard.2014.10.032","volume":"115","author":"I Lindsay","year":"2015","unstructured":"Lindsay I, Johnson J, Everitt MD, Hoffman J, Yetman AT. Impact of liver disease after the Fontan operation. Am J Cardiol. 2015;115:249\u201352.","journal-title":"Am J Cardiol"},{"key":"2378_CR31","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1016\/j.amjcard.2015.11.014","volume":"117","author":"K Pundi","year":"2016","unstructured":"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\u201360.","journal-title":"Am J Cardiol"},{"key":"2378_CR32","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.116.004809","volume":"6","author":"DJ Goldberg","year":"2017","unstructured":"Goldberg DJ, Surrey LF, Glatz AC, Dodds K, O\u2019Byrne ML, Lin HC, et al. Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: a liver biopsy and hemodynamic study. J Am Heart Assoc. 2017;6:6.","journal-title":"J Am Heart Assoc"},{"key":"2378_CR33","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1007\/s00246-020-02291-5","volume":"41","author":"A Schleiger","year":"2020","unstructured":"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\u201346.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR34","doi-asserted-by":"publisher","first-page":"1750","DOI":"10.1136\/hrt.2010.201772","volume":"96","author":"JS Baek","year":"2010","unstructured":"Baek JS, Bae EJ, Ko JS, Kim GB, Kwon BS, Lee SY, et al. Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors. Heart. 2010;96:1750\u20135.","journal-title":"Heart"},{"key":"2378_CR35","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1016\/j.healun.2013.10.033","volume":"33","author":"KE Simpson","year":"2014","unstructured":"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\u20137.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR36","doi-asserted-by":"publisher","first-page":"1348","DOI":"10.1016\/j.jtcvs.2004.10.005","volume":"129","author":"AA Ghaferi","year":"2005","unstructured":"Ghaferi AA, Hutchins GM. Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg. 2005;129:1348\u201352.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR37","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1136\/hrt.2006.094516","volume":"93","author":"CH Kiesewetter","year":"2007","unstructured":"Kiesewetter CH, Sheron N, Vettukattill JJ, Hacking N, Stedman B, Millward-Sadler H, et al. Hepatic changes in the failing Fontan circulation. Heart. 2007;93:579\u201384.","journal-title":"Heart"},{"key":"2378_CR38","doi-asserted-by":"publisher","first-page":"1335","DOI":"10.1016\/j.dld.2023.12.017","volume":"56","author":"C Jarasvaraparn","year":"2024","unstructured":"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. Dig Liver Dis. 2024;56:1335\u201342.","journal-title":"Dig Liver Dis"},{"key":"2378_CR39","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2021.100362","volume":"3","author":"J Emamaullee","year":"2021","unstructured":"Emamaullee J, Khan S, Weaver C, Goldbeck C, Yanni G, Kohli R, et al. Non-invasive biomarkers of Fontan-associated liver disease. JHEP Rep. 2021;3:100362.","journal-title":"JHEP Rep"},{"issue":"1","key":"2378_CR40","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1007\/s12072-025-10819-1","volume":"20","author":"C Jarasvaraparn","year":"2025","unstructured":"Jarasvaraparn C, Rodenbarger A, Thoe J, Vuppalanchi R, Payne RM, Markham LW, et al. Platelet slope and long-term clinical outcomes in children and adults with Fontan-associated liver disease. Hepatol Int. 2025;20(1):174\u201385.","journal-title":"Hepatol Int"},{"key":"2378_CR41","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.121.024034","volume":"12","author":"CE Guerrero-Chalela","year":"2023","unstructured":"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.","journal-title":"J Am Heart Assoc"},{"key":"2378_CR42","doi-asserted-by":"publisher","first-page":"276","DOI":"10.1136\/heartjnl-2022-320940","volume":"109","author":"R Inuzuka","year":"2023","unstructured":"Inuzuka R, Nii M, Inai K, Shimada E, Shinohara T, Kogiso T, et al. Predictors of liver cirrhosis and hepatocellular carcinoma among perioperative survivors of the Fontan operation. Heart. 2023;109:276\u201382.","journal-title":"Heart"},{"key":"2378_CR43","doi-asserted-by":"publisher","first-page":"664","DOI":"10.1016\/j.athoracsur.2022.06.018","volume":"115","author":"TG Wilson","year":"2023","unstructured":"Wilson TG, Iyengar AJ, Zentner D, Zannino D, d\u2019Udekem Y, Konstantinov IE. Liver cirrhosis after the Fontan procedure: impact of atrioventricular valve failure. Ann Thorac Surg. 2023;115:664\u201370.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR44","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1111\/chd.12446","volume":"12","author":"MB Hilscher","year":"2017","unstructured":"Hilscher MB, Johnson JN, Cetta F, Driscoll DJ, Poterucha JJ, Sanchez W, et al. Surveillance for liver complications after the Fontan procedure. Congenit Heart Dis. 2017;12:124\u201332.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR45","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1055\/s-0031-1284434","volume":"20","author":"AM Alvarez","year":"2011","unstructured":"Alvarez AM, Mukherjee D. Liver abnormalities in cardiac diseases and heart failure. Int J Angiol. 2011;20:135\u201342.","journal-title":"Int J Angiol"},{"key":"2378_CR46","doi-asserted-by":"publisher","first-page":"3764","DOI":"10.1016\/j.ijcard.2013.06.008","volume":"168","author":"RW Elder","year":"2013","unstructured":"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\u20139.","journal-title":"Int J Cardiol"},{"key":"2378_CR47","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1111\/j.1747-0803.2012.00632.x","volume":"7","author":"S Ginde","year":"2012","unstructured":"Ginde S, Hohenwalter MD, Foley WD, Sowinski J, Bartz PJ, Venkatapuram S, et al. Noninvasive assessment of liver fibrosis in adult patients following the Fontan procedure. Congenit Heart Dis. 2012;7:235\u201342.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR48","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1016\/j.ijcard.2016.02.041","volume":"209","author":"G Agnoletti","year":"2016","unstructured":"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\u20135.","journal-title":"Int J Cardiol"},{"key":"2378_CR49","doi-asserted-by":"publisher","first-page":"1043","DOI":"10.1002\/hep.26643","volume":"59","author":"AQ Farias","year":"2014","unstructured":"Farias AQ, Silvestre OM, Garcia-Tsao G, da Costa Seguro LF, de Campos Mazo DF, Bacal F, et al. Serum B-type natriuretic peptide in the initial workup of patients with new onset ascites: a diagnostic accuracy study. Hepatology. 2014;59:1043\u201351.","journal-title":"Hepatology"},{"key":"2378_CR50","doi-asserted-by":"publisher","first-page":"1300","DOI":"10.1002\/hep.32406","volume":"75","author":"MB Hilscher","year":"2022","unstructured":"Hilscher MB, Wells ML, Venkatesh SK, Cetta F, Kamath PS. Fontan-associated liver disease. Hepatology. 2022;75:1300\u201321.","journal-title":"Hepatology"},{"key":"2378_CR51","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1161\/CIRCULATIONAHA.120.045597","volume":"142","author":"J Emamaullee","year":"2020","unstructured":"Emamaullee J, Zaidi AN, Schiano T, Kahn J, Valentino PL, Hofer RE, et al. Fontan-associated liver disease: screening, management, and transplant considerations. Circulation. 2020;142:591\u2013604.","journal-title":"Circulation"},{"key":"2378_CR52","doi-asserted-by":"publisher","first-page":"1552","DOI":"10.1038\/modpathol.2014.79","volume":"27","author":"DF Dai","year":"2014","unstructured":"Dai DF, 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\u20138.","journal-title":"Mod Pathol"},{"key":"2378_CR53","doi-asserted-by":"publisher","first-page":"766","DOI":"10.1097\/PAS.0000000000001250","volume":"43","author":"DE Bosch","year":"2019","unstructured":"Bosch DE, Koro K, Richards E, Hoch BL, Jalikis F, Koch LK, et al. Validation of a congestive hepatic fibrosis scoring system. Am J Surg Pathol. 2019;43:766\u201372.","journal-title":"Am J Surg Pathol"},{"key":"2378_CR54","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1016\/j.humpath.2016.07.006","volume":"57","author":"LF Surrey","year":"2016","unstructured":"Surrey LF, Russo P, Rychik J, Goldberg DJ, Dodds K, O\u2019Byrne ML, et al. Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation. Hum Pathol. 2016;57:106\u201315.","journal-title":"Hum Pathol"},{"key":"2378_CR55","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/j.ijcard.2020.11.037","volume":"328","author":"AA Borquez","year":"2021","unstructured":"Borquez AA, Silva-Sepulveda J, Lee JW, Vavinskaya V, Vodkin I, El-Sabrout H, et al. Transjugular liver biopsy for Fontan associated liver disease surveillance: technique, outcomes and hemodynamic correlation. Int J Cardiol. 2021;328:83\u20138.","journal-title":"Int J Cardiol"},{"key":"2378_CR56","doi-asserted-by":"publisher","first-page":"1057","DOI":"10.1093\/eurheartj\/ehy620","volume":"40","author":"ID Munsterman","year":"2019","unstructured":"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\u201368.","journal-title":"Eur Heart J"},{"key":"2378_CR57","doi-asserted-by":"publisher","first-page":"600","DOI":"10.1111\/chd.12770","volume":"14","author":"JA Silva-Sepulveda","year":"2019","unstructured":"Silva-Sepulveda JA, Fonseca Y, Vodkin I, Vaughn G, Newbury R, Vavinskaya V, et al. Evaluation of Fontan liver disease: correlation of transjugular liver biopsy with magnetic resonance and hemodynamics. Congenit Heart Dis. 2019;14:600\u20138.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR58","doi-asserted-by":"publisher","first-page":"883","DOI":"10.1016\/j.healun.2015.01.993","volume":"34","author":"FM Wu","year":"2015","unstructured":"Wu FM, Jonas MM, Opotowsky AR, Harmon A, Raza R, Ukomadu C, et al. Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes. J Heart Lung Transplant. 2015;34:883\u201391.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR59","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1007\/s00247-018-4311-9","volume":"49","author":"A Srinivasan","year":"2019","unstructured":"Srinivasan A, Guzman AK, Rand EB, Rychik J, Goldberg DJ, Russo PA, et al. Percutaneous liver biopsy in Fontan patients. Pediatr Radiol. 2019;49:342\u201350.","journal-title":"Pediatr Radiol"},{"key":"2378_CR60","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.14120","volume":"34","author":"SS Vaikunth","year":"2020","unstructured":"Vaikunth SS, Higgins JP, Concepcion W, Haeffele C, Wright GE, Chen S, et al. Does liver biopsy accurately measure fibrosis in Fontan-associated liver disease? A comparison of liver biopsy pre-combined heart and liver transplant and liver explant post-transplant. Clin Transplant. 2020;34:e14120.","journal-title":"Clin Transplant"},{"key":"2378_CR61","doi-asserted-by":"publisher","first-page":"1270","DOI":"10.1016\/j.jhep.2023.07.013","volume":"79","author":"L T\u00e9llez","year":"2023","unstructured":"T\u00e9llez L, Payanc\u00e9 A, Tjwa E, Del Cerro MJ, Idorn L, Ovroutski S, et al. EASL-ERN position paper on liver involvement in patients with Fontan-type circulation. J Hepatol. 2023;79:1270\u2013301.","journal-title":"J Hepatol"},{"key":"2378_CR62","doi-asserted-by":"publisher","first-page":"656","DOI":"10.1016\/j.jtcvs.2016.10.060","volume":"153","author":"FM Wu","year":"2017","unstructured":"Wu FM, Kogon B, Earing MG, Aboulhosn JA, Broberg CS, John AS, et al. Liver health in adults with Fontan circulation: a multicenter cross-sectional study. J Thorac Cardiovasc Surg. 2017;153:656\u201364.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR63","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1002\/cld.676","volume":"10","author":"A Lemmer","year":"2017","unstructured":"Lemmer A, VanWagner L, Ganger D. Congestive hepatopathy: differentiating congestion from fibrosis. Clin Liver Dis (Hoboken). 2017;10:139\u201343.","journal-title":"Clin Liver Dis (Hoboken)"},{"key":"2378_CR64","doi-asserted-by":"publisher","first-page":"1528","DOI":"10.1007\/s00247-020-04776-0","volume":"50","author":"JR Dillman","year":"2020","unstructured":"Dillman JR, Trout AT, Alsaied T, Gupta A, Lubert AM. Imaging of Fontan-associated liver disease. Pediatr Radiol. 2020;50:1528\u201341.","journal-title":"Pediatr Radiol"},{"key":"2378_CR65","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacadv.2024.101357","volume":"3","author":"ML Garagiola","year":"2024","unstructured":"Garagiola ML, Tan SB, Alonso-Gonzalez R, O\u2019Brien CM. Liver imaging in Fontan patients: how does ultrasound compare to cross-sectional imaging? JACC Adv. 2024;3:101357.","journal-title":"JACC Adv"},{"key":"2378_CR66","doi-asserted-by":"publisher","first-page":"850","DOI":"10.1016\/j.ejrad.2016.02.002","volume":"85","author":"JM Bae","year":"2016","unstructured":"Bae JM, Jeon TY, Kim JS, Kim S, Hwang SM, Yoo SY, et al. Fontan-associated liver disease: spectrum of US findings. Eur J Radiol. 2016;85:850\u20136.","journal-title":"Eur J Radiol"},{"key":"2378_CR67","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1007\/s00431-021-04163-3","volume":"181","author":"R De Bruyne","year":"2022","unstructured":"De Bruyne R, Vandekerckhove K, Van Overschelde H, Hendricx F, Vande Walle C, De Groote K, et al. Non-invasive assessment of liver abnormalities in pediatric Fontan patients. Eur J Pediatr. 2022;181:159\u201369.","journal-title":"Eur J Pediatr"},{"key":"2378_CR68","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1097\/RCT.0000000000001533","volume":"48","author":"MJ Brown","year":"2024","unstructured":"Brown MJ, Kolbe AB, Hull NC, Hilscher M, Kamath PS, Yalon M, et al. Imaging of Fontan-associated liver disease. J Comput Assist Tomogr. 2024;48:1\u201311.","journal-title":"J Comput Assist Tomogr"},{"key":"2378_CR69","doi-asserted-by":"publisher","first-page":"3205","DOI":"10.1007\/s00261-021-02994-0","volume":"46","author":"KJ Thrane","year":"2021","unstructured":"Thrane KJ, M\u00fcller LSO, Suther KR, Thomassen KS, Holmstr\u00f6m H, Thaulow E, et al. Spectrum of Fontan-associated liver disease assessed by MRI and US in young adolescents. Abdom Radiol (NY). 2021;46:3205\u201316.","journal-title":"Abdom Radiol (NY)"},{"key":"2378_CR70","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1016\/j.ijcard.2015.09.088","volume":"202","author":"D Wolff","year":"2016","unstructured":"Wolff D, van Melle JP, Dijkstra H, Bartelds B, Willems TP, Hillege H, et al. The Fontan circulation and the liver: a magnetic resonance diffusion-weighted imaging study. Int J Cardiol. 2016;202:595\u2013600.","journal-title":"Int J Cardiol"},{"key":"2378_CR71","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1007\/s00247-021-05221-6","volume":"52","author":"M Navallas","year":"2022","unstructured":"Navallas M, Yoo SJ, Chavhan GB, Amirabadi A, Ling SC, Seed M, et al. Semiquantitative characterization of dynamic magnetic resonance perfusion of the liver in pediatric Fontan patients. Pediatr Radiol. 2022;52:483\u201392.","journal-title":"Pediatr Radiol"},{"key":"2378_CR72","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2019.6762","volume":"2","author":"Z Mandalenakis","year":"2019","unstructured":"Mandalenakis Z, Karazisi C, Skoglund K, Rosengren A, Lappas G, Eriksson P, et al. Risk of cancer among children and young adults with congenital heart disease compared with healthy controls. JAMA Netw Open. 2019;2:e196762.","journal-title":"JAMA Netw Open"},{"key":"2378_CR73","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1136\/heartjnl-2022-321435","volume":"109","author":"I Martin de Miguel","year":"2023","unstructured":"Martin de Miguel I, Kamath PS, Egbe AC, Jain CC, Cetta F, Connolly HM, et al. Haemodynamic and prognostic associations of liver fibrosis scores in Fontan-associated liver disease. Heart. 2023;109:619\u201325.","journal-title":"Heart"},{"key":"2378_CR74","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezaf100","volume":"67","author":"S Horie","year":"2025","unstructured":"Horie S, Shikata F, Oka N, Okamura T, Matsunaga Y, Matsui K, et al. Liver fibrosis marker is a potential predictor of the development of Fontan-associated liver diseases\u2020. Eur J Cardiothorac Surg. 2025;67:67.","journal-title":"Eur J Cardiothorac Surg"},{"key":"2378_CR75","doi-asserted-by":"publisher","DOI":"10.1097\/HC9.0000000000000580","volume":"8","author":"JH Seol","year":"2024","unstructured":"Seol JH, Song J, Kim SJ, Ko H, Na JY, Cho MJ, et al. Clinical predictors and noninvasive imaging in Fontan-associated liver disease: a systematic review and meta-analysis. Hepatol Commun. 2024;8:8.","journal-title":"Hepatol Commun"},{"key":"2378_CR76","doi-asserted-by":"publisher","first-page":"1274","DOI":"10.1007\/s00246-016-1428-1","volume":"37","author":"WN Evans","year":"2016","unstructured":"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\u20137.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR77","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1002\/lt.20906","volume":"13","author":"DM Heuman","year":"2007","unstructured":"Heuman DM, Mihas AA, Habib A, Gilles HS, Stravitz RT, Sanyal AJ, et al. MELD-XI: a rational approach to \u201csickest first\u201d liver transplantation in cirrhotic patients requiring anticoagulant therapy. Liver Transpl. 2007;13:30\u20137.","journal-title":"Liver Transpl"},{"key":"2378_CR78","doi-asserted-by":"crossref","unstructured":"Grimm JC, Shah AS, Magruder JT, Kilic A, Valero V 3rd, Dungan SP, et al. MELD-XI score predicts early mortality in patients after heart transplantation. Ann Thorac Surg. 2015;100:1737\u201343.","DOI":"10.1016\/j.athoracsur.2015.07.026"},{"key":"2378_CR79","doi-asserted-by":"publisher","first-page":"730","DOI":"10.1016\/j.athoracsur.2015.06.063","volume":"101","author":"JC Grimm","year":"2016","unstructured":"Grimm JC, Magruder JT, Do N, Spinner JA, Dungan SP, Kilic A, et al. Modified Model for End-Stage Liver Disease eXcluding INR (MELD-XI) score predicts early death after pediatric heart transplantation. Ann Thorac Surg. 2016;101:730\u20135.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR80","doi-asserted-by":"publisher","first-page":"1462","DOI":"10.1016\/j.jtcvs.2021.02.014","volume":"163","author":"S Amdani","year":"2022","unstructured":"Amdani S, Simpson KE, Thrush P, Shih R, Simmonds J, Knecht K, et al. Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients. J Thorac Cardiovasc Surg. 2022;163:1462-1473.e1412.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR81","doi-asserted-by":"publisher","first-page":"593","DOI":"10.1111\/hepr.13627","volume":"51","author":"Y Cho","year":"2021","unstructured":"Cho Y, Kabata D, Ehara E, Yamamoto A, Mizuochi T, Mushiake S, et al. Assessing liver stiffness with conventional cut-off values overestimates liver fibrosis staging in patients who received the Fontan procedure. Hepatol Res. 2021;51:593\u2013602.","journal-title":"Hepatol Res"},{"key":"2378_CR82","doi-asserted-by":"publisher","first-page":"1454","DOI":"10.1016\/j.amjcard.2019.07.052","volume":"124","author":"T Alsaied","year":"2019","unstructured":"Alsaied T, Possner M, Lubert AM, Trout AT, Szugye C, Palermo JJ, et al. Relation of magnetic resonance elastography to Fontan failure and portal hypertension. Am J Cardiol. 2019;124:1454\u20139.","journal-title":"Am J Cardiol"},{"key":"2378_CR83","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.23.29640","volume":"222","author":"BE Derinkuyu","year":"2024","unstructured":"Derinkuyu BE, Dillman JR, Lubert AM, Palermo JJ, Opotowsky AR, Trout AT. Associations of liver stiffness measured by ultrasound shear-wave elastography with portal hypertension and circulatory failure in individuals with Fontan circulation. AJR Am J Roentgenol. 2024;222:e2329640.","journal-title":"AJR Am J Roentgenol"},{"key":"2378_CR84","doi-asserted-by":"publisher","first-page":"1328","DOI":"10.1002\/jmri.24496","volume":"40","author":"DB Wallihan","year":"2014","unstructured":"Wallihan DB, Podberesky DJ, Marino BS, Sticka JS, Serai S. Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation. J Magn Reson Imaging. 2014;40:1328\u201335.","journal-title":"J Magn Reson Imaging"},{"key":"2378_CR85","doi-asserted-by":"publisher","first-page":"e776","DOI":"10.1016\/j.crad.2022.06.016","volume":"77","author":"SD Serai","year":"2022","unstructured":"Serai SD, Tsitsiou Y, Wilkins BJ, Ghosh A, Cahill AM, Biko DM, et al. MR elastography-based staging of liver fibrosis in Fontan procedure associated liver disease is confounded by effects of venous congestion. Clin Radiol. 2022;77:e776\u201382.","journal-title":"Clin Radiol"},{"key":"2378_CR86","doi-asserted-by":"publisher","first-page":"764","DOI":"10.1111\/chd.12650","volume":"13","author":"JL Schachter","year":"2018","unstructured":"Schachter JL, Patel M, Horton SR, Devane MA, Ewing A, Abrams GA. FibroSURE and elastography poorly predict the severity of liver fibrosis in Fontan-associated liver disease. Congenit Heart Dis. 2018;13:764\u201370.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR87","doi-asserted-by":"publisher","first-page":"1871","DOI":"10.1007\/s00246-021-02707-w","volume":"42","author":"SW Brayer","year":"2021","unstructured":"Brayer SW, Zafar F, Lubert AM, Trout AT, Palermo JJ, Opotowsky AR, et al. Relation of magnetic resonance elastography to Fontan circulatory failure in a cohort of pediatric and adult patients. Pediatr Cardiol. 2021;42:1871\u20138.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR88","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1002\/hep.26631","volume":"59","author":"SS Kutty","year":"2014","unstructured":"Kutty SS, Peng Q, Danford DA, Fletcher SE, Perry D, Talmon GA, et al. Increased hepatic stiffness as consequence of high hepatic afterload in the Fontan circulation: a vascular Doppler and elastography study. Hepatology. 2014;59:251\u201360.","journal-title":"Hepatology"},{"key":"2378_CR89","doi-asserted-by":"publisher","first-page":"299","DOI":"10.1016\/j.ijcard.2018.01.108","volume":"258","author":"A Egbe","year":"2018","unstructured":"Egbe A, Miranda WR, Connolly HM, Khan AR, Al-Otaibi M, Venkatesh SK, et al. Temporal changes in liver stiffness after Fontan operation: results of serial magnetic resonance elastography. Int J Cardiol. 2018;258:299\u2013304.","journal-title":"Int J Cardiol"},{"key":"2378_CR90","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v31.i11.103178","volume":"31","author":"M Cuadros","year":"2025","unstructured":"Cuadros M, Abad\u00eda M, Castillo P, Mart\u00edn-Arranz MD, Gonzalo N, Romero M, et al. Role of transient elastography in the diagnosis and prognosis of Fontan-associated liver disease. World J Gastroenterol. 2025;31:103178.","journal-title":"World J Gastroenterol"},{"key":"2378_CR91","doi-asserted-by":"publisher","first-page":"480","DOI":"10.1016\/j.jhep.2024.09.005","volume":"82","author":"L T\u00e9llez","year":"2025","unstructured":"T\u00e9llez L, Rinc\u00f3n D, Payanc\u00e9 A, Jaillais A, Lebray P, Rodr\u00edguez de Santiago E, et al. Non-invasive assessment of severe liver fibrosis in patients with Fontan-associated liver disease: the VALDIG-EASL FONLIVER cohort. J Hepatol. 2025;82:480\u20139.","journal-title":"J Hepatol"},{"key":"2378_CR92","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1111\/chd.12730","volume":"14","author":"EM Engelhardt","year":"2019","unstructured":"Engelhardt EM, Trout AT, Sheridan RM, Veldtman GR, Dillman JR. Focal liver lesions following Fontan palliation of single ventricle physiology: a radiology-pathology case series. Congenit Heart Dis. 2019;14:380\u20138.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR93","doi-asserted-by":"publisher","first-page":"702","DOI":"10.1016\/j.jhep.2019.10.027","volume":"72","author":"L T\u00e9llez","year":"2020","unstructured":"T\u00e9llez L, Rodr\u00edguez de Santiago E, Minguez B, Payance A, Clemente A, Baiges A, et al. Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: the VALDIG Fonliver prospective cohort. J Hepatol. 2020;72:702\u201310.","journal-title":"J Hepatol"},{"key":"2378_CR94","doi-asserted-by":"publisher","first-page":"882","DOI":"10.1016\/j.mayocp.2015.04.020","volume":"90","author":"JT Poterucha","year":"2015","unstructured":"Poterucha JT, Johnson JN, Qureshi MY, O\u2019Leary PW, Kamath PS, Lennon RJ, et al. Magnetic resonance elastography: a novel technique for the detection of hepatic fibrosis and hepatocellular carcinoma after the Fontan operation. Mayo Clin Proc. 2015;90:882\u201394.","journal-title":"Mayo Clin Proc"},{"key":"2378_CR95","doi-asserted-by":"publisher","first-page":"201","DOI":"10.1016\/j.jpeds.2012.12.071","volume":"163","author":"OP Bulut","year":"2013","unstructured":"Bulut OP, Romero R, Mahle WT, McConnell M, Braithwaite K, Shehata BM, et al. Magnetic resonance imaging identifies unsuspected liver abnormalities in patients after the Fontan procedure. J Pediatr. 2013;163:201\u20136.","journal-title":"J Pediatr"},{"key":"2378_CR96","doi-asserted-by":"publisher","first-page":"746","DOI":"10.1161\/CIRCULATIONAHA.117.032717","volume":"138","author":"AC Egbe","year":"2018","unstructured":"Egbe AC, Poterucha JT, Warnes CA, Connolly HM, Baskar S, Ginde S, et al. Hepatocellular carcinoma after Fontan operation: multicenter case series. Circulation. 2018;138:746\u20138.","journal-title":"Circulation"},{"key":"2378_CR97","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1016\/j.ijcard.2020.08.057","volume":"322","author":"M Possner","year":"2021","unstructured":"Possner M, Gordon-Walker T, Egbe AC, Poterucha JT, Warnes CA, Connolly HM, et al. Hepatocellular carcinoma and the Fontan circulation: clinical presentation and outcomes. Int J Cardiol. 2021;322:142\u20138.","journal-title":"Int J Cardiol"},{"key":"2378_CR98","doi-asserted-by":"publisher","first-page":"2623","DOI":"10.1007\/s00261-017-1181-9","volume":"42","author":"ML Wells","year":"2017","unstructured":"Wells ML, Hough DM, Fidler JL, Kamath PS, Poterucha JT, Venkatesh SK. Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma. Abdom Radiol (NY). 2017;42:2623\u201331.","journal-title":"Abdom Radiol (NY)"},{"key":"2378_CR99","doi-asserted-by":"publisher","first-page":"268","DOI":"10.1016\/j.ijcard.2010.05.047","volume":"151","author":"T Bryant","year":"2011","unstructured":"Bryant T, Ahmad Z, Millward-Sadler H, Burney K, Stedman B, Kendall T, et al. Arterialised hepatic nodules in the Fontan circulation: hepatico-cardiac interactions. Int J Cardiol. 2011;151:268\u201372.","journal-title":"Int J Cardiol"},{"key":"2378_CR100","doi-asserted-by":"publisher","DOI":"10.1148\/rg.240063","volume":"45","author":"AB Kolbe","year":"2025","unstructured":"Kolbe AB, Acord MR, Khanna G, Morin CE, Nguyen HN, Rees MA, et al. Imaging findings and management strategies for liver masses in children with predisposition disorders: a review by the Pediatric LI-RADS Group. Radiographics. 2025;45:e240063.","journal-title":"Radiographics"},{"key":"2378_CR101","doi-asserted-by":"publisher","first-page":"188","DOI":"10.1007\/s00261-019-02119-8","volume":"45","author":"HK Yang","year":"2020","unstructured":"Yang HK, Jang HJ, Khalili K, Wald RM, Yoo SJ, Kim TK. CT and MR imaging findings of the livers in adults with Fontan palliation: an observational study. Abdom Radiol (NY). 2020;45:188\u2013202.","journal-title":"Abdom Radiol (NY)"},{"key":"2378_CR102","doi-asserted-by":"publisher","first-page":"816","DOI":"10.1148\/radiol.2018181494","volume":"289","author":"V Chernyak","year":"2018","unstructured":"Chernyak V, Fowler KJ, Kamaya A, Kielar AZ, Elsayes KM, Bashir MR, et al. Liver Imaging Reporting and Data System (LI-RADS) version 2018: imaging of hepatocellular carcinoma in at-risk patients. Radiology. 2018;289:816\u201330.","journal-title":"Radiology"},{"key":"2378_CR103","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1111\/hepr.13582","volume":"51","author":"E Rodriguez De Santiago","year":"2021","unstructured":"Rodriguez De Santiago E, T\u00e9llez L, Guerrero A, Albillos A. Hepatocellular carcinoma after Fontan surgery: a systematic review. Hepatol Res. 2021;51:116\u201334.","journal-title":"Hepatol Res"},{"key":"2378_CR104","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0257441","volume":"16","author":"Y Isoura","year":"2021","unstructured":"Isoura Y, Yamamoto A, Cho Y, Ehara E, Jogo A, Suzuki T, et al. Platelet count and abdominal dynamic CT are useful in predicting and screening for gastroesophageal varices after Fontan surgery. PLoS ONE. 2021;16:e0257441.","journal-title":"PLoS ONE"},{"key":"2378_CR105","doi-asserted-by":"crossref","unstructured":"Liu X, Han L, Zhou Z, Tu J, Ma J, Chen J. Effect of liver abnormalities on mortality in Fontan patients: a systematic review and meta-analysis. BMC Cardiovasc Disord. 2024;24:385.","DOI":"10.1186\/s12872-024-04042-3"},{"key":"2378_CR106","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacadv.2025.101646","volume":"4","author":"BE Rosenthal","year":"2025","unstructured":"Rosenthal BE, Hoteit MA, Lluri G, Haeffele C, Daugherty T, Krasuski RA, et al. Characteristics and survival outcomes of hepatocellular carcinoma after the Fontan operation. JACC Adv. 2025;4:101646.","journal-title":"JACC Adv"},{"key":"2378_CR107","doi-asserted-by":"publisher","first-page":"3173","DOI":"10.1016\/j.jacc.2017.10.045","volume":"70","author":"CJ Daniels","year":"2017","unstructured":"Daniels CJ, Bradley EA, Landzberg MJ, Aboulhosn J, Beekman RH 3rd, Book W, et al. Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015 Washington DC. J Am Coll Cardiol. 2017;70:3173\u201394.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR108","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1161\/CIRCULATIONAHA.105.548016","volume":"114","author":"D Bernstein","year":"2006","unstructured":"Bernstein D, Naftel D, Chin C, Addonizio LJ, Gamberg P, Blume ED, et al. Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study. Circulation. 2006;114:273\u201380.","journal-title":"Circulation"},{"key":"2378_CR109","doi-asserted-by":"publisher","first-page":"1315","DOI":"10.1016\/j.athoracsur.2016.08.110","volume":"103","author":"KE Simpson","year":"2017","unstructured":"Simpson KE, Pruitt E, Kirklin JK, Naftel DC, Singh RK, Edens RE, et al. Fontan patient survival after pediatric heart transplantation has improved in the current era. Ann Thorac Surg. 2017;103:1315\u201320.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR110","doi-asserted-by":"publisher","first-page":"823","DOI":"10.1016\/j.athoracsur.2010.11.031","volume":"91","author":"KR Kanter","year":"2011","unstructured":"Kanter KR, Mahle WT, Vincent RN, Berg AM, Kogon BE, Kirshbom PM. Heart transplantation in children with a Fontan procedure. Ann Thorac Surg. 2011;91:823\u20139.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR111","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.jpeds.2020.09.044","volume":"229","author":"DS Rodriguez","year":"2021","unstructured":"Rodriguez DS, Mao C, Mahle WT, Kanter KR, Alazraki A, Braithwaite K, et al. Pretransplantation and post-transplantation liver disease assessment in adolescents undergoing isolated heart transplantation for Fontan failure. J Pediatr. 2021;229:78-85.e72.","journal-title":"J Pediatr"},{"key":"2378_CR112","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.117.003754","volume":"11","author":"J Bouchardy","year":"2018","unstructured":"Bouchardy J, Meyer P, Yerly P, Blanche C, Hullin R, Giostra E, et al. Regression of advanced liver fibrosis after heart transplantation in a patient with prior Fontan surgery for complex congenital heart disease. Circ Heart Fail. 2018;11:e003754.","journal-title":"Circ Heart Fail"},{"key":"2378_CR113","doi-asserted-by":"publisher","DOI":"10.1111\/petr.14094","volume":"25","author":"AM Ybarra","year":"2021","unstructured":"Ybarra AM, Khanna G, Turmelle YP, Stoll J, Castleberry CD, Scheel J, et al. Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure. Pediatr Transplant. 2021;25:e14094.","journal-title":"Pediatr Transplant"},{"key":"2378_CR114","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.13540","volume":"33","author":"SS Vaikunth","year":"2019","unstructured":"Vaikunth SS, Concepcion W, Daugherty T, Fowler M, Lutchman G, Maeda K, et al. Short-term outcomes of en bloc combined heart and liver transplantation in the failing Fontan. Clin Transplant. 2019;33:e13540.","journal-title":"Clin Transplant"},{"key":"2378_CR115","doi-asserted-by":"publisher","first-page":"3522","DOI":"10.1111\/ajt.13870","volume":"16","author":"TW Wong","year":"2016","unstructured":"Wong TW, Gandhi MJ, Daly RC, Kushwaha SS, Pereira NL, Rosen CB, et al. Liver allograft provides immunoprotection for the cardiac allograft in combined heart-liver transplantation. Am J Transplant. 2016;16:3522\u201331.","journal-title":"Am J Transplant"},{"key":"2378_CR116","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.12892","volume":"31","author":"BA D\u2019Souza","year":"2017","unstructured":"D\u2019Souza BA, Fuller S, Gleason LP, Hornsby N, Wald J, Krok K, et al. Single-center outcomes of combined heart and liver transplantation in the failing Fontan. Clin Transplant. 2017;31:e12892.","journal-title":"Clin Transplant"},{"key":"2378_CR117","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1016\/j.healun.2020.12.008","volume":"40","author":"D Sganga","year":"2021","unstructured":"Sganga D, Hollander SA, Vaikunth S, Haeffele C, Bensen R, Navaratnam M, et al. Comparison of combined heart-liver vs heart-only transplantation in pediatric and young adult Fontan recipients. J Heart Lung Transplant. 2021;40:298\u2013306.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR118","doi-asserted-by":"publisher","first-page":"858","DOI":"10.1016\/j.athoracsur.2014.04.100","volume":"98","author":"P Atluri","year":"2014","unstructured":"Atluri P, Gaffey A, Howard J, Phillips E, Goldstone AB, Hornsby N, et al. Combined heart and liver transplantation can be safely performed with excellent short- and long-term results. Ann Thorac Surg. 2014;98:858\u201362.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR119","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1097\/TP.0b013e3181ac60db","volume":"88","author":"E Raichlin","year":"2009","unstructured":"Raichlin E, Daly RC, Rosen CB, McGregor CG, Charlton MR, Frantz RP, et al. Combined heart and liver transplantation: a single-center experience. Transplantation. 2009;88:219\u201325.","journal-title":"Transplantation"},{"key":"2378_CR120","doi-asserted-by":"publisher","first-page":"290","DOI":"10.1002\/lt.20360","volume":"11","author":"F Bettens","year":"2005","unstructured":"Bettens F, Tiercy JM, Campanile N, Giostra E, Majno P, Rubbia L, et al. Microchimerism after liver transplantation: absence of rejection without abrogation of anti-donor cytotoxic T-lymphocyte-mediated alloreactivity. Liver Transpl. 2005;11:290\u20137.","journal-title":"Liver Transpl"},{"key":"2378_CR121","doi-asserted-by":"publisher","first-page":"e553","DOI":"10.1161\/CIR.0000000000000598","volume":"139","author":"MM Colvin","year":"2019","unstructured":"Colvin MM, Cook JL, Chang PP, Hsu DT, Kiernan MS, Kobashigawa JA, et al. Sensitization in heart transplantation: emerging knowledge: a scientific statement from the American Heart Association. Circ. 2019;139:e553\u201378.","journal-title":"Circ"},{"key":"2378_CR122","doi-asserted-by":"publisher","first-page":"2161","DOI":"10.1016\/j.jacc.2023.03.422","volume":"81","author":"MJ Lewis","year":"2023","unstructured":"Lewis MJ, Reardon LC, Aboulhosn J, Haeffele C, Chen S, Kim Y, et al. Morbidity and mortality in adult Fontan patients after heart or combined heart-liver transplantation. J Am Coll Cardiol. 2023;81:2161\u201371.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR123","doi-asserted-by":"publisher","first-page":"2149","DOI":"10.1016\/j.jacc.2023.03.421","volume":"81","author":"MJ Lewis","year":"2023","unstructured":"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\u201360.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR124","doi-asserted-by":"publisher","first-page":"e67","DOI":"10.1097\/TP.0000000000001978","volume":"102","author":"R Bryant 3rd","year":"2018","unstructured":"Bryant R 3rd, Rizwan R, Zafar F, Shah SA, Chin C, Tweddell JS, et al. Contemporary outcomes of combined heart-liver transplant in patients with congenital heart disease. Transplant. 2018;102:e67\u201373.","journal-title":"Transplant"},{"key":"2378_CR125","doi-asserted-by":"publisher","first-page":"1462","DOI":"10.1016\/j.jtcvs.2021.02.014","volume":"163","author":"S Amdani","year":"2022","unstructured":"Amdani S, Simpson KE, Thrush P, Shih R, Simmonds J, Knecht K, et al. Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients. J Thorac Cardiovasc Surg. 2022;163:1462-1473.e1412.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR126","doi-asserted-by":"publisher","first-page":"222","DOI":"10.1016\/j.healun.2015.10.008","volume":"35","author":"SV Deo","year":"2016","unstructured":"Deo SV, Al-Kindi SG, Altarabsheh SE, Hang D, Kumar S, Ginwalla MB, et al. Model for end-stage liver disease excluding international normalized ratio (MELD-XI) score predicts heart transplant outcomes: evidence from the registry of the United Network for Organ Sharing. J Heart Lung Transplant. 2016;35:222\u20137.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR127","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1111\/j.1747-0803.2007.00116.x","volume":"2","author":"J Rychik","year":"2007","unstructured":"Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis. 2007;2:288\u2013300.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR128","doi-asserted-by":"publisher","first-page":"2929","DOI":"10.1016\/j.jacc.2017.04.023","volume":"69","author":"M Itkin","year":"2017","unstructured":"Itkin M, Piccoli DA, Nadolski G, Rychik J, DeWitt A, Pinto E, et al. Protein-losing enteropathy in patients with congenital heart disease. J Am Coll Cardiol. 2017;69:2929\u201337.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR129","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1017\/S1047951111000102","volume":"21","author":"J Meadows","year":"2011","unstructured":"Meadows J, Jenkins K. Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment. Cardiol Young. 2011;21:363\u201377.","journal-title":"Cardiol Young"},{"key":"2378_CR130","doi-asserted-by":"publisher","first-page":"886","DOI":"10.1007\/s00246-017-1576-y","volume":"38","author":"S Menon","year":"2017","unstructured":"Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS. The Lymphatic Circulation in Adaptations to the Fontan Circulation. Pediatr Cardiol. 2017;38:886\u201392.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR131","doi-asserted-by":"crossref","unstructured":"Simpson DE. No central venous pressure protein losing enteropathy relation? Blame the albumin. Eur J Cardiothorac Surg. 2014;46:146.","DOI":"10.1093\/ejcts\/ezt506"},{"key":"2378_CR132","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1136\/hrt.79.3.213","volume":"79","author":"K Bull","year":"1998","unstructured":"Bull K. The Fontan procedure: lessons from the past. Heart. 1998;79:213\u20134.","journal-title":"Heart"},{"key":"2378_CR133","doi-asserted-by":"publisher","first-page":"7809","DOI":"10.1074\/jbc.M510722200","volume":"281","author":"L Bode","year":"2006","unstructured":"Bode L, Murch S, Freeze HH. Heparan Sulfate Plays a Central Role in a Dynamic in Vitro Model of Protein-losing Enteropathy*. J Biol Chem. 2006;281:7809\u201315.","journal-title":"J Biol Chem"},{"key":"2378_CR134","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1016\/j.athoracsur.2006.02.048","volume":"82","author":"AM Ostrow","year":"2006","unstructured":"Ostrow AM, Freeze H, Rychik J. Protein-Losing Enteropathy After Fontan Operation: Investigations Into Possible Pathophysiologic Mechanisms. Ann Thorac Surg. 2006;82:695\u2013700.","journal-title":"Ann Thorac Surg"},{"key":"2378_CR135","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1007\/s00246-017-1796-1","volume":"39","author":"C Miranda","year":"2018","unstructured":"Miranda C, Taqatqa A, Chapa-Rodriguez A, Holton JP, Awad SM. The Use of Fecal Calprotectin Levels in the Fontan population. Pediatr Cardiol. 2018;39:591\u20134.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR136","doi-asserted-by":"publisher","first-page":"2158","DOI":"10.1016\/j.jtcvs.2020.07.107","volume":"161","author":"VJ Sharma","year":"2021","unstructured":"Sharma VJ, Iyengar AJ, Zannino D, Gentles T, Justo R, Celermajer DS, et al. Protein-losing enteropathy and plastic bronchitis after the Fontan procedure. J Thorac Cardiovasc Surg. 2021;161:2158-2165.e2154.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR137","doi-asserted-by":"publisher","first-page":"672","DOI":"10.1016\/S0002-9149(02)02584-5","volume":"90","author":"J Rychik","year":"2002","unstructured":"Rychik J, Gui-Yang S. Relation of mesenteric vascular resistance after Fontan operation and protein-losing enteropathy. Am J Cardiol. 2002;90:672\u20134.","journal-title":"Am J Cardiol"},{"key":"2378_CR138","doi-asserted-by":"publisher","first-page":"1063","DOI":"10.1016\/S0022-5223(98)70406-4","volume":"115","author":"L Mertens","year":"1998","unstructured":"Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. J Thorac Cardiovasc Surg. 1998;115:1063\u201373.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR139","doi-asserted-by":"publisher","first-page":"970","DOI":"10.1016\/j.jpeds.2014.12.068","volume":"166","author":"KR Schumacher","year":"2015","unstructured":"Schumacher KR, Stringer KA, Donohue JE, Yu S, Shaver A, Caruthers RL, et al. Fontan-associated protein-losing enteropathy and plastic bronchitis. J Pediatr. 2015;166:970\u20137.","journal-title":"J Pediatr"},{"key":"2378_CR140","doi-asserted-by":"publisher","first-page":"e49","DOI":"10.1093\/ejcts\/ezs714","volume":"43","author":"H Ohuchi","year":"2013","unstructured":"Ohuchi H, Yasuda K, Miyazaki A, Kitano M, Sakaguchi H, Yazaki S, et al. Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation. Eur J Cardiothorac Surg. 2013;43:e49-57.","journal-title":"Eur J Cardiothorac Surg"},{"key":"2378_CR141","volume":"7","author":"T Alsaied","year":"2022","unstructured":"Alsaied T, Lubert AM, Goldberg DJ, Schumacher K, Rathod R, Katz DA, et al. Protein losing enteropathy after the Fontan operation. Int J Cardiol Congenit Heart Dis. 2022;7:100338.","journal-title":"Int J Cardiol Congenit Heart Dis"},{"key":"2378_CR142","volume":"7","author":"R Barracano","year":"2022","unstructured":"Barracano R, Merola A, Fusco F, Scognamiglio G, Sarubbi B. Protein-losing enteropathy in Fontan circulation: pathophysiology, outcome and treatment options of a complex condition. Int J Cardiol Congenit Heart Dis. 2022;7:100322.","journal-title":"Int J Cardiol Congenit Heart Dis"},{"key":"2378_CR143","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.119.015318","volume":"9","author":"RM Ghosh","year":"2020","unstructured":"Ghosh RM, Griffis HM, Glatz AC, Rome JJ, Smith CL, Gillespie MJ, et al. Prevalence and cause of early Fontan complications: Does the lymphatic circulation play a role? J Am Heart Assoc. 2020;9:e015318.","journal-title":"J Am Heart Assoc"},{"key":"2378_CR144","doi-asserted-by":"publisher","first-page":"426","DOI":"10.2214\/AJR.13.11797","volume":"203","author":"Y Dori","year":"2014","unstructured":"Dori Y, Keller MS, Fogel MA, Rome JJ, Whitehead KK, Harris MA, et al. MRI of lymphatic abnormalities after functional single-ventricle palliation surgery. AJR Am J Roentgenol. 2014;203:426\u201331.","journal-title":"AJR Am J Roentgenol"},{"key":"2378_CR145","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1177\/2150135120905656","volume":"11","author":"C Kreutzer","year":"2020","unstructured":"Kreutzer C, Klinger DA, Chiostri B, Sendoya S, Daneri ML, Gutierrez A, et al. Lymphatic decompression concomitant with Fontan\/Kreutzer procedure: early experience. World J Pediatr Congenit Heart Surg. 2020;11:284\u201392.","journal-title":"World J Pediatr Congenit Heart Surg"},{"key":"2378_CR146","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1053\/pcsu.2002.31498","volume":"5","author":"J Rychik","year":"2002","unstructured":"Rychik J, Spray TL. Strategies to treat protein-losing enteropathy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002;5:3\u201311.","journal-title":"Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu"},{"key":"2378_CR147","doi-asserted-by":"publisher","first-page":"966","DOI":"10.1007\/s00246-011-0029-2","volume":"32","author":"KR Schumacher","year":"2011","unstructured":"Schumacher KR, Cools M, Goldstein BH, Ioffe-Dahan V, King K, Gaffney D, et al. Oral budesonide treatment for protein-losing enteropathy in Fontan-palliated patients. Pediatr Cardiol. 2011;32:966\u201371.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR148","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1007\/s00246-019-02248-3","volume":"41","author":"RO Roberts","year":"2020","unstructured":"Roberts RO, Di Maria MV, Brigham D, Hsu S. Evidence of systemic absorption of enteral budesonide in patients with Fontan-associated protein-losing enteropathy. Pediatr Cardiol. 2020;41:241\u201350.","journal-title":"Pediatr Cardiol"},{"key":"2378_CR149","doi-asserted-by":"publisher","first-page":"248","DOI":"10.1016\/j.amjcard.2007.08.029","volume":"101","author":"L Ryerson","year":"2008","unstructured":"Ryerson L, Goldberg C, Rosenthal A, Armstrong A. Usefulness of heparin therapy in protein-losing enteropathy associated with single ventricle palliation. Am J Cardiol. 2008;101:248\u201351.","journal-title":"Am J Cardiol"},{"key":"2378_CR150","doi-asserted-by":"publisher","first-page":"653","DOI":"10.1111\/j.1747-0803.2011.00518.x","volume":"6","author":"AS John","year":"2011","unstructured":"John AS, Phillips SD, Driscoll DJ, Warnes CA, Cetta F. The use of octreotide to successfully treat protein-losing enteropathy following the Fontan operation. Congenit Heart Dis. 2011;6:653\u20136.","journal-title":"Congenit Heart Dis"},{"key":"2378_CR151","doi-asserted-by":"publisher","first-page":"784","DOI":"10.1016\/j.healun.2019.04.002","volume":"38","author":"AJ Weingarten","year":"2019","unstructured":"Weingarten AJ, Menachem JN, Smith CA, Frischhertz BP, Book WM. Usefulness of midodrine in protein-losing enteropathy. J Heart Lung Transplant. 2019;38:784\u20137.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR152","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12925","volume":"21","author":"JM Friedland-Little","year":"2017","unstructured":"Friedland-Little JM, Gajarski RJ, Schumacher KR. Dopamine as a potential rescue therapy for refractory protein-losing enteropathy in Fontan-palliated patients. Pediatr Transplant. 2017;21:e12925.","journal-title":"Pediatr Transplant"},{"key":"2378_CR153","first-page":"E56","volume":"95","author":"CL Smith","year":"2020","unstructured":"Smith CL, Hoffman TM, Dori Y, Rome JJ. Decompression of the thoracic duct: a novel transcatheter approach. Catheter Cardiovasc Interv. 2020;95:E56-e61.","journal-title":"Catheter Cardiovasc Interv"},{"key":"2378_CR154","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/j.xjtc.2021.01.045","volume":"7","author":"V Hraska","year":"2021","unstructured":"Hraska V, Hjortdal VE, Dori Y, Kreutzer C. Innominate vein turn-down procedure: killing two birds with one stone. JTCVS Tech. 2021;7:253\u201360.","journal-title":"JTCVS Tech"},{"key":"2378_CR155","first-page":"849","volume":"86","author":"A Hallbergson","year":"2015","unstructured":"Hallbergson A, Mascio CE, Rome JJ. Transcatheter Fontan takedown. Catheter Cardiovasc Interv. 2015;86:849\u201354.","journal-title":"Catheter Cardiovasc Interv"},{"key":"2378_CR156","doi-asserted-by":"publisher","first-page":"1126","DOI":"10.1016\/j.jtcvs.2020.09.074","volume":"161","author":"SP Marathe","year":"2021","unstructured":"Marathe SP, Iyengar AJ, Betts KS, du Plessis K, Salve GG, Justo RN, et al. Long-term outcomes following Fontan takedown in Australia and New Zealand. J Thorac Cardiovasc Surg. 2021;161:1126\u201335.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR157","doi-asserted-by":"publisher","first-page":"698","DOI":"10.1017\/S1047951120000864","volume":"30","author":"A Schleiger","year":"2020","unstructured":"Schleiger A, Ovroutski S, Peters B, Schubert S, Photiadis J, Berger F, et al. Treatment strategies for protein-losing enteropathy in Fontan-palliated patients. Cardiol Young. 2020;30:698\u2013709.","journal-title":"Cardiol Young"},{"key":"2378_CR158","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.healun.2018.09.024","volume":"38","author":"KR Schumacher","year":"2019","unstructured":"Schumacher KR, Yu S, Butts R, Castleberry C, Chen S, Edens E, et al. Fontan-associated protein-losing enteropathy and post-heart transplant outcomes: a multicenter study. J Heart Lung Transplant. 2019;38:17\u201325.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR159","doi-asserted-by":"publisher","first-page":"1221","DOI":"10.1016\/j.healun.2011.06.005","volume":"30","author":"WT Mahle","year":"2011","unstructured":"Mahle WT, Tresler MA, Edens RE, Rusconi P, George JF, Naftel DC, et al. Allosensitization and outcomes in pediatric heart transplantation. J Heart Lung Transplant. 2011;30:1221\u20137.","journal-title":"J Heart Lung Transplant"},{"key":"2378_CR160","doi-asserted-by":"publisher","first-page":"672","DOI":"10.1016\/S0022-5223(96)70051-X","volume":"112","author":"RH Feldt","year":"1996","unstructured":"Feldt RH, Driscoll DJ, Offord KP, Cha RH, Perrault J, Schaff HV, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996;112:672\u201380.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2378_CR161","doi-asserted-by":"publisher","first-page":"54","DOI":"10.1016\/j.jacc.2014.04.025","volume":"64","author":"AS John","year":"2014","unstructured":"John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol. 2014;64:54\u201362.","journal-title":"J Am Coll Cardiol"},{"key":"2378_CR162","doi-asserted-by":"publisher","first-page":"632","DOI":"10.1007\/s00246-013-0831-0","volume":"35","author":"BW McCrindle","year":"2014","unstructured":"McCrindle BW, Zak V, Breitbart RE, Mahony L, Shrader P, Lai WW, et al. The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure. Pediatr Cardiol. 2014;35:632\u201340.","journal-title":"Pediatr Cardiol"}],"container-title":["Current Cardiology Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11886-026-02378-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11886-026-02378-0","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11886-026-02378-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T10:02:33Z","timestamp":1779184953000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s11886-026-02378-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,19]]},"references-count":162,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["2378"],"URL":"https:\/\/doi.org\/10.1007\/s11886-026-02378-0","relation":{},"ISSN":["1534-3170"],"issn-type":[{"value":"1534-3170","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,19]]},"assertion":[{"value":"18 February 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 May 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 May 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"No financial relationships relevant to this article to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Financial Disclosure"}},{"value":"This article does not contain any studies with human or animal subjects performed by any of the authors.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Human and Animal Rights and Informed Consent"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}}],"article-number":"56"}}