Int J Cardiol Congenit Heart Dis. 2026 Jun 22;25:100693. doi: 10.1016/j.ijcchd.2026.100693. eCollection 2026 Sep.
ABSTRACT
BACKGROUND: Fontan associated liver disease (FALD) is a known complication in adult patients with total cavo-pulmonary connection. The relationship between the severity of FALD and cardiovascular disease progression is yet to be clarified. We investigated the role of liver stiffness measured by transient elastography (TE) in predicting adverse events (AEs) in adult patients with Fontan circulation.
MATERIAL AND METHODS: We retrospectively analysed TE data from our cohort of Fontan patients from September 2014 to December 2023. Baseline characteristics were recorded. The association of TE, liver function tests, and pro-collagen3 with AEs were analysed.
RESULTS: 167/207 patients (median age 20.0 years; 60% male) for a total of 613 TE from September 2014 to December 2023 (median follow up 56.0 months; 1.893 patient-year) were included in the study. TE values remained stable over time and did not show any correlation with baseline characteristics. TE values < 23.9 kPa could discriminate patients with advanced FALD (Se 50%; Sp 79%; NPV 97%). Forty AEs occurred (0.06 events per patient-year). Albumin (HR 0.91, p = 0.010) and APRI (HR 1.92, p = 0.034) were independent predictors of morbidity at multivariate analysis.
CONCLUSIONS: In this relatively young cohort, with low mortality and no HCC during follow-up, no significant association was found between TE and AEs. Nevertheless, TE appeared to be useful in discriminating advanced FALD. As no convincing risk factor related to FALD severity or progression has been yet identified, a multidisciplinary approach to risk assessment and surveillance is advised to reduce the impact of FALD and prevent progression to HCC.
PMID:42434196 | PMC:PMC13351556 | DOI:10.1016/j.ijcchd.2026.100693