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

Montanaro, C.; Tamborrino, PP.; Verma, S.; Butera, G.; Dimopoulos, K.; Ladouceur, M.; Veldtman, G.; Baessato, F.; Rocafort, AG.; Ponz de Antonio, I.; Pandja, B.; Frigiola, A.; Iannaccone, G.; Kacar, P.; English, K.; Prokselj, K.; Carbonell, M.; van de Bruaene, A.; Giannakoulas, G.; Jenkins, P.; Sarris, G.; Kansy, A.; Roos-Hesselink, JW.; Payance, A.; Fraga, M.; Bouchardy, J.; Voges, I.; Tutarel, O.; Ovaert, C.; Jalal, Z.; Chemello, L.; Padalino, MA.; Tritto, G.; Gatzoulis, MA.

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

All Authors

Montanaro, C.

Tamborrino, PP.

Verma, S.

Butera, G.

Dimopoulos, K.

Ladouceur, M.

Veldtman, G.

Baessato, F.

Rocafort, AG.

Ponz de Antonio, I.

Show 10 more

LTHT Author

English, Kate

LTHT Department

Cardio-Respiratory
Cardiology
Congenital Cardiac Services

Publication Date

2025

Item Type

Journal Article

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.