{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T20:30:43Z","timestamp":1789072243911,"version":"build-2803163510"},"reference-count":28,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,9,23]],"date-time":"2021-09-23T00:00:00Z","timestamp":1632355200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 \u00b1 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan\u00ae). According to selective criteria for liver disease, we identified 23\/43 (53.5%) cases with advanced FALD that showed: older age (p &lt; 0.05), larger hepatic and cava veins diameter (p &lt; 0.05), worsened NYHA class (p &lt; 0.05), abnormal lymphocytes (p &lt; 0.01), platelet count (p &lt; 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (p &lt; 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (p &lt; 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (p &lt; 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD.<\/jats:p>","DOI":"10.3390\/jcdd8100117","type":"journal-article","created":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T00:30:33Z","timestamp":1632443433000},"page":"117","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction"],"prefix":"10.3390","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6584-4157","authenticated-orcid":false,"given":"Liliana","family":"Chemello","sequence":"first","affiliation":[{"name":"Clinica Medica 5, Internal Medicine & Hepatology Unit, Department of Medicine-DIMED, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7535-9670","authenticated-orcid":false,"given":"Massimo","family":"Padalino","sequence":"additional","affiliation":[{"name":"Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic & Vascular Sciences and Public Health, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Zanon","sequence":"additional","affiliation":[{"name":"Clinica Medica 5, Internal Medicine & Hepatology Unit, Department of Medicine-DIMED, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luisa","family":"Benvegnu\u2019","sequence":"additional","affiliation":[{"name":"Clinica Medica 5, Internal Medicine & Hepatology Unit, Department of Molecular Medicine, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Biffanti","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Unit, Department of Woman\u2019s and Child\u2019s Health, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Mancuso","sequence":"additional","affiliation":[{"name":"Cardiologic Unit, Department of Cardiac, Thoracic & Vascular Sciences and Public Health, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7368-279X","authenticated-orcid":false,"given":"Luisa","family":"Cavalletto","sequence":"additional","affiliation":[{"name":"Clinica Medica 5, Internal Medicine & Hepatology Unit, Department of Medicine-DIMED, University of Padua Medical School, 35128 Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,9,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1161\/01.CIR.31.2.172","article-title":"Circulatory Bypass of the Right Side of the Heart. Vi. Shunt between Superior Vena Cava and Distal Right Pulmonary Artery; Report of Clinical Application in Thirty-Eight Cases","volume":"31","author":"Glenn","year":"1965","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/0002-9149(80)90224-6","article-title":"Hypoplastic left heart syndrome: Experience with palliative surgery","volume":"45","author":"Norwood","year":"1980","journal-title":"Am. J. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1136\/hrt.2004.051789","article-title":"The Fontan circulation","volume":"91","author":"Gewillig","year":"2005","journal-title":"Heart"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"192","DOI":"10.5604\/01.3001.0010.8634","article-title":"Fontan-Associated Liver Disease: A Review","volume":"17","author":"Albillos","year":"2018","journal-title":"Ann. Hepatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3173","DOI":"10.1016\/j.jacc.2017.10.045","article-title":"Fontan-Associated Liver Disease","volume":"70","author":"Daniels","year":"2017","journal-title":"JACC"},{"key":"ref_6","first-page":"850","article-title":"Fontan-associated liver disease: Spectrum of US findings","volume":"7","author":"Bea","year":"2016","journal-title":"Eur. J. Radiol."},{"key":"ref_7","first-page":"100249","article-title":"From congestive hepatopathy to hepatocellular carcinoma, how can we to improve the management of the patients?","volume":"27","author":"Sessa","year":"2021","journal-title":"J. Hep. Rep."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1093\/icvts\/ivw186","article-title":"Liver stiffness modifications shortly after total cavopulmonary connection","volume":"23","author":"Deorsola","year":"2016","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1148\/rg.312105027","article-title":"The Fontan procedure: Anatomy, complications, and manifestations of failure","volume":"31","author":"Fredenburg","year":"2011","journal-title":"RadioGraphics"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1007\/s00246-020-02291-5","article-title":"Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics","volume":"41","author":"Schleiger","year":"2020","journal-title":"Pediatr. Cardiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1136\/heartjnl-2011-301133","article-title":"Management of failing Fontan circulation","volume":"98","author":"Deal","year":"2012","journal-title":"Heart"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1136\/jcp.2007.052365","article-title":"Hepatic fibrosis and cirrhosis in the Fontan circulation: A detailed morphological study","volume":"61","author":"Kendall","year":"2007","journal-title":"J. Clin. Pathol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/j.humpath.2016.07.006","article-title":"Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation","volume":"57","author":"Surrey","year":"2016","journal-title":"Hum. Pathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1382","DOI":"10.1038\/ajg.2009.750","article-title":"A Liver Stiffness Measurement-Based, Noninvasive Prediction Model for High-Risk Esophageal Varices in B-Viral Liver Cirrhosis","volume":"105","author":"Kim","year":"2010","journal-title":"Am. J. Gastroenterol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"15","DOI":"10.3389\/fcvm.2017.00015","article-title":"Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients","volume":"4","author":"Fidai","year":"2017","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Padalino, M.A., Chemello, L., Cavalletto, L., Angelini, A., and Fedrigo, M. (2021). Prognostic value of liver and spleen stiffness in patients with Fontan Associated Liver Disease (FALD): A Case Series with Histopathologic Comparison. JCDD, 8.","DOI":"10.3390\/jcdd8030030"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1053\/jhep.2003.50346","article-title":"A simple noninvasive index can predict both significant fibrosis and cirrhosis in patients with chronic hepatitis C","volume":"38","author":"Wai","year":"2003","journal-title":"Hepatology"},{"key":"ref_18","first-page":"986","article-title":"Identification of chronic hepatitis C patients without hepatic fibrosis by a simple predictive model","volume":"36","author":"Forns","year":"2002","journal-title":"Hepatology"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1002\/hep.21178","article-title":"Development of a simple noninvasive index to predict significant fibrosis in patients with HIV\/HCV coinfection","volume":"43","author":"Sterling","year":"2006","journal-title":"Hepatology"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1016\/j.athoracsur.2015.06.063","article-title":"Modified Model for End-Stage Liver Disease eXcluding INR (MELD-XI) Score Predicts Early Death After Pediatric Heart Transplantation","volume":"101","author":"Grimm","year":"2016","journal-title":"Ann. Thorac. Surg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1080\/17474124.2019.1621746","article-title":"Liver disease secondary to congenital heart disease in children","volume":"13","author":"Komatsu","year":"2019","journal-title":"Expert Rev. Gastroenterol. Hepatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e234","DOI":"10.1161\/CIR.0000000000000696","article-title":"Evaluation and Management of the Child and Adult with Fontan Circulation: A Scientific Statement from the American Heart Association. A Scientific statement from the AHA","volume":"140","author":"Rychik","year":"2019","journal-title":"Circulation"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1093\/ejcts\/ezaa310","article-title":"Evaluation of Fontan failure by classifying the severity of Fontan-associated liver disease: A single-centre cross-sectional study","volume":"59","author":"Schleiger","year":"2021","journal-title":"Eur. J. Cardio-Torac. Surg."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1016\/j.jtcvs.2016.10.060","article-title":"Liver health in adults with Fontan circulation: A multicenter cross-sectional study","volume":"153","author":"Wu","year":"2017","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1161\/JAHA.116.004809","article-title":"Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated with Time from Surgery: A Liver Biopsy and Hemodynamic Study","volume":"6","author":"Goldberg","year":"2017","journal-title":"J. Am. Heart Assoc."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1007\/s13244-018-0609-2","article-title":"Abdominal findings in adult patients with Fontan circulation","volume":"9","author":"Kim","year":"2018","journal-title":"Insights Imaging"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1016\/j.jhep.2011.09.024","article-title":"Clinical evidence of regression of liver fibrosis","volume":"56","author":"Ellis","year":"2012","journal-title":"J. Hepatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1111\/j.1432-2277.2005.00122.x","article-title":"Cardiac hepatopathy before and after heart transplantation","volume":"18","author":"Dichtl","year":"2005","journal-title":"Transpl. 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