Liver health in adults with Fontan circulation: A multicenter cross-sectional study

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 153; no. 3; pp. 656 - 664
• Main Authors: Wu, Fred M., Kogon, Brian, Earing, Michael G., Aboulhosn, Jamil A., Broberg, Craig S., John, Anitha S., Harmon, Amy, Sainani, Nisha I., Hill, Andrew J., Odze, Robert D., Johncilla, Melanie E., Ukomadu, Chinweike, Gauvreau, Kimberlee, Valente, Anne Marie, Landzberg, Michael J.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.03.2017
• Subjects: Adolescent; Adult; adult congenital heart disease; Biopsy; Cardiothoracic Surgery; Child; congenital heart disease; congestive hepatopathy; Cross-Sectional Studies; Female; Fontan circulation; Fontan Procedure - adverse effects; Heart Defects, Congenital - surgery; hepatic fibrosis; Humans; Liver - pathology; Liver Cirrhosis - diagnosis; Liver Cirrhosis - epidemiology; Liver Cirrhosis - etiology; Liver Function Tests; Male; Middle Aged; Prevalence; Prognosis; Risk Factors; Severity of Illness Index; Time Factors; United States - epidemiology; Young Adult; United States; AARCC; AST; MELD-XI; NAFLD; RV; MRI; PLE; ALT; HCC; IQR; INR; LLN; RA; CT; PA; congestive hepatopathy; adult congenital heart disease; GGT; ULN; congenital heart disease; Fontan circulation; hepatic fibrosis; magnetic resonance imaging; pulmonary artery; alanine aminotransferase; model for end-stage liver disease, excluding INR; lower limit of normal; interquartile range; international normalized ratio; Alliance for Adult Research in Congenital Cardiology; computerized tomography; hepatocellular carcinoma; protein-losing enteropathy; aspartate aminotransferase; nonalcoholic fatty liver disease; upper limit of normal; right ventricle; right atrium; gamma glutamyl transpeptidase
• ISSN: 0022-5223; 1097-685X; 1097-685X
• DOI: 10.1016/j.jtcvs.2016.10.060

Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Of 241 subjects (median age 25.8 years [11.8-59.4], median time since Fontan 20.3 years [5.4-34.5]), more than 94% of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33% of histology specimens. Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population.