TY - JOUR
T1 - Live(r) with a Fontan circulation
T2 - A European survey and a proposed expert consensus on liver surveillance in collaboration with the EuroFontan group
AU - Montanaro, Claudia
AU - Tamborrino, Pietro Paolo
AU - Verma, Suman
AU - Butera, Gianfranco
AU - Dimopoulos, Konstantinos
AU - Ladouceur, Magalie
AU - Veldtman, Gruschen
AU - Baessato, Francesca
AU - Rocafort, Alvaro Gonzalez
AU - Ponz de Antonio, Inés
AU - Pandja, Bejal
AU - Frigiola, Alessandra
AU - Iannaccone, Giulia
AU - Kacar, Polona
AU - English, Kate
AU - Prokselj, Katja
AU - Carbonell, Michael
AU - van de Bruaene, Alexander
AU - Giannakoulas, George
AU - Jenkins, Petra
AU - Sarris, Georges
AU - Kansy, Andrzej
AU - Roos-Hesselink, Jolien W.
AU - Payance, Audrey
AU - Fraga, Montserrat
AU - Bouchardy, Judith
AU - Voges, Inga
AU - Tutarel, Oktay
AU - Ovaert, Caroline
AU - Jalal, Zakaria
AU - Chemello, Liliana
AU - Padalino, Massimo A.
AU - Tritto, Giovanni
AU - Gatzoulis, Michael A.
N1 - Publisher Copyright:
© 2025
PY - 2026/3/15
Y1 - 2026/3/15
N2 - 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.
AB - 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.
UR - https://www.scopus.com/pages/publications/105027448245
U2 - 10.1016/j.ijcard.2025.134118
DO - 10.1016/j.ijcard.2025.134118
M3 - Article
C2 - 41455559
AN - SCOPUS:105027448245
SN - 0167-5273
VL - 447
JO - International Journal of Cardiology
JF - International Journal of Cardiology
M1 - 134118
ER -