Live(r) with a Fontan circulation: A European survey and a proposed expert consensus on liver surveillance in collaboration with the EuroFontan group

Abstract

BACKGROUND: A widely accepted protocol for the diagnosis and follow-up of Fontan Associated Liver Disease (FALD) is currently lacking, thus we undertook a European survey with the dual aim of examining the current status quo and developing a shared screening algorithm. METHODS: In this cross-sectional qualitative study, 39 Adult Congenital Heart Disease (ACHD) centres in Europe were invited (between August 2023 and April 2024) to participate in a survey regarding their approach to FALD diagnosis and surveillance. RESULTS: Twenty-two (54.5 %) centres from 11 Countries responded to the survey. Patients with a Fontan circulation had annual liver assessment in the majority of centres, with review of selected case by a specialist hepatologist. Different scoring systems were employed to quantify the severity of FALD (Child-Pugh score, MELD XI, FIB4). Ultrasound was the first-line imaging modality, followed by magnetic resonance imaging and computed tomography. Liver fibrosis and portal hypertension were assessed with different modalities and at variable time intervals ranging from 1 to 3 years, with the most used technique being the Fibroscan (74 %). Half of the centres would consider transplant referral in the presence of FALD. Combined heart-liver transplantation was an option in 8 centres. CONCLUSIONS: Our data suggests universal agreement amongst European ACHD centres on the need for regular assessment of adult patients for FALD, often with the involvement of a hepatologist, though the approaches varied widely between centres. We propose herewith a FALD surveillance algorithm to introduce a uniform approach to this complex entity which impacts on the growing number of patients with a Fontan circulation.