Live(r) with a Fontan circulation: A European survey and a proposed expert consensus on liver surveillance in collaboration with the EuroFontan group.
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Montanaro Claudia, Tamborrino Pietro Paolo, Verma Suman, Butera Gianfranco, Dimopoulos Konstantinos, Ladouceur Magalie, Veldtman Gruschen, Baessato Francesca, Rocafort Alvaro Gonzalez, Ponz de Antonio Inés, Pandja Bejal, Frigiola Alessandra, Iannaccone Giulia, Kacar Polona, English Kate, Prokselj Katja, Carbonell Michael, van de Bruaene Alexander, Giannakoulas George, Jenkins Petra, Sarris Georges, Kansy Andrzej, Roos-Hesselink Jolien W, Payance Audrey, Fraga Montserrat, Bouchardy Judith, Voges Inga, Tutarel Oktay, Ovaert Caroline, Jalal Zakaria, Chemello Liliana, Padalino Massimo A, Tritto Giovanni, Gatzoulis Michael A
Аннотация
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.