Determinants of Advanced Liver Fibrosis in Adult Patients After Fontan Palliation: Usefulness of Ultrasound Transient Elastography.

Autor: Bütikofer S; Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland., Greutmann-Yantiri M; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Gubler C; Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland., Reiner C; Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Alkadhi H; Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Pfammatter T; Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Puippe G; Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Santos Lopes B; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Possner M; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Bonassin F; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Meier L; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Babic D; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Attenhofer Jost C; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland., Jüngst C; Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland., Müllhaupt B; Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland., Bernsmeier C; Department of Gastroenterology and Hepatology, University Hospital Basel, Basel, Switzerland., Schwerzmann M; Center for Congenital Heart Disease, University Hospital Inselspital, University of Bern, Bern, Switzerland., Tobler D; Department of Cardiology, University Hospital Basel, Basel, Switzerland., Lenggenhager D; Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, Switzerland., Marques Maggio E; Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, Switzerland., Greutmann M; University Heart Centre Zurich, University Hospital Zurich, University of Zurich, Zurich, Switzerland. Electronic address: Matthias.greutmann@usz.ch.
Jazyk: angličtina
Zdroj: The Canadian journal of cardiology [Can J Cardiol] 2023 Oct; Vol. 39 (10), pp. 1338-1345. Date of Electronic Publication: 2023 Apr 26.
DOI: 10.1016/j.cjca.2023.04.019
Abstrakt: Background: Fontan-associated liver disease is an increasing concern. Our aim was to assess prevalence and predictors of advanced liver fibrosis with a specific focus on utility of liver stiffness measurement by ultrasound transient elastography.
Methods: A total of 97 adult Fontan patients (55% male, median age: 23.1 years, interquartile range [IQR]: 18.7-30.6); 92 (95%) were evaluated with transient elastography, and 50 (52%) underwent transjugular liver biopsy. Advanced liver fibrosis was defined as congestive hepatic fibrosis score 3 or 4.
Results: Only 4 patients (4%) had liver stiffness values < 10 kilopascal (kPa). Liver-stiffness measurements correlated weakly with peak oxygen uptake on exercise testing and Fontan pressure but not with Model for End-Stage Liver Disease excluding INR (MELD-XI) score or spleen size. Serial follow-up liver stiffness measurements in 73 clinically stable patients showed large variability among individual patients. Advanced liver fibrosis was present in 35 of 50 (70%) patients on liver biopsy and was associated to MELD-XI-Score ≥ 11 and splenomegaly but not to liver-stiffness measurements. Advanced liver fibrosis was not associated with patient age or time since Fontan operation but with younger age at completion of Fontan (3.7 years, IQR: 2.3-6.3 vs 6.8 years; IQR: 3.5-12.1; P = 0.037).
Conclusions: In our cohort, advanced liver fibrosis was present in the majority of adult Fontan patients. Liver stiffness as measured by transient elastography was not associated with the degree of liver fibrosis. Because of its high variability on serial measurements, it seems not to be useful for clinical decision making. The unexpected finding that younger age at completion of Fontan was associated with advanced liver fibrosis merits further evaluation.
(Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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