Congenital heart diseaseCohort study

Differences in Prevalence and Severity of Liver Disease Between Lateral Tunnel and Extracardiac Conduit Fontan

JACC AdvPublished

  • retrospective study

Authors' conclusion

ECC 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.

Study context

Population
Adults with total cavopulmonary connection (TCPC), in a primary-TCPC cohort (TCPC at the initial Fontan operation) and an FC-TCPC cohort (Fontan conversion to TCPC)
Exposure
extracardiac conduit vs lateral tunnel

Main finding

As reported in the abstract

Prevalence of cirrhosis (primary-TCPC cohort) · baseline

P = 0.03

Observational data: associations, not effects.

Further findings

OutcomeValuesP value
APRI (primary-TCPC cohort) · baseline · worse disease severity with ECCECC 0.46 (range as printed [0.34;0.61]) · LT 0.39 (range as printed [0.29;0.52])P < 0.001
FIB-4 (primary-TCPC cohort) · baseline · worse disease severity with ECCECC 0.89 (range as printed [0.59;1.12]) · LT 0.76 (range as printed [0.31;0.99])P = 0.009

Liver disease progression (primary-TCPC cohort)

Both groups had progression of liver disease; Extracardiac conduit (ECC) group had a greater temporal increase in biomarker levels, with a higher relative increase in model for end-stage liver disease excluding international normalized ratio, FIB-4, and APRI

Prevalence, severity and progression of liver disease (FC-TCPC group)

No significant differences between ECC and lateral tunnel (LT)

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Differences in Prevalence and Severity of Liver Disease Between Lateral Tunnel and Extracardiac Conduit Fontan. JACC Adv. Published 17 March 2026. doi:10.1016/j.jacadv.2026.102680

Summarised from the published abstract; every number is checked against it