Determinants of advanced liver fibrosis in adult patients after Fontan-palliation: Usefulness of ultrasound transient elastography.

Bütikofer, Simon; Greutmann-Yantiri, Mehtap; Gubler, Christoph; Reiner, Cecilia; Alkadhi, Hatem; Pfammatter, Thomas; Puippe, Gilbert; Santos Lopes, Bruno; Possner, Mathias; Bonassin, Francesca; Meier, Lukas; Babic, Daniela; Attenhofer Jost, Christine; Jüngst, Christoph; Müllhaupt, Beat; Bernsmeier, Christine; Schwerzmann, Markus; Tobler, Daniel; Lenggenhager, Daniela; Marques Maggio, Ewerton; ... (2023). Determinants of advanced liver fibrosis in adult patients after Fontan-palliation: Usefulness of ultrasound transient elastography. Canadian journal of cardiology, 39(10), pp. 1338-1345. Elsevier 10.1016/j.cjca.2023.04.019

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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

97 adult Fontan-patients (55% males, median age: 23.1 years, 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 four patients (4%) had liver stiffness values <10kPa. 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 35/50 (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 Fontan-completion (3.7 years, IQR: 2.3-6.3 versus 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. Due to its high variability on serial measurements it seems not to be useful for clinical decision-making. The unexpected finding that younger age at Fontan-completion was associated with advanced liver fibrosis merits further evaluation.

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