ArticleJACC. Advances2026
Differences in Prevalence and Severity of Liver Disease Between Lateral Tunnel and Extracardiac Conduit Fontan.
Article in JACC. Advances, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- Limited association between liver stiffness and clinical outcomes in Fontan-associated liver disease: a retrospective analysis.International journal of cardiology. Congenital heart disease · 2026Article
- Cross-Sectional Area Changes in the Total Cavopulmonary Connection Pathway and Its Impact on Liver Fibrosis.Journal of clinical medicine · 2026Article
- The Fontan circulation: Arrhythmia is just one price we have to pay.Annals of pediatric cardiologyArticle
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundRecent studies suggest differences in liver disease severity based on the type of total cavopulmonary connection (TCPC).
objectivesThe purpose of this study was to compare the severity and progression of liver disease between patients with extracardiac conduit (ECC) vs lateral tunnel (LT) using 2 separate cohorts: i) TCPC at the initial Fontan operation (primary-TCPC cohort) and ii) Fontan conversion (FC) to TCPC (FC-TCPC cohort).
methodThis is a retrospective study of adults with TCPC (ECC [N = 62, 46%]; LT [N = 73,54%). Liver disease severity was assessed at baseline using biomarkers (model for end-stage liver disease excluding international normalized ratio, Fibrosis-4 [FIB-4], aspartate aminotransferase to platelet ratio index [APRI]), and progression of liver disease was assessed as temporal change in biomarkers at 3, 5, and 7 years.
resultsIn the primary-TCPC cohort (ECC [N = 62,46%]; LT [N = 73,54%), patients with ECC had a higher prevalence of cirrhosis (27% vs 12%; P = 0.03) and worse disease severity at baseline (APRI 0.46 [0.34;0.61] vs 0.39 [0.29;0.52], P < 0.001; FIB-4 (0.89 [0.59;1.12] vs 0.76 [0.31;0.99], P = 0.009) compared to LT. Both groups had progression of liver disease (temporal increase in biomarker levels), but the ECC group had a greater temporal increase in biomarker levels resulting in a higher relative increase in model for end-stage liver disease excluding international normalized ratio, FIB-4, and APRI. There were no significant differences in prevalence and severity of liver disease at baseline, or liver disease progression during follow-up between patients with ECC vs LT in the FC-TCPC group.
conclusionsECC was associated with a higher prevalence and worse severity of liver disease at baseline, and greater disease progression during follow-up compared to those with LT. Further studies are required to determine the optimal strategies for the management of liver disease in patients with ECC.
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