Evaluation of Fontan failure by classifying the severity of Fontan-associated liver disease: a single-centre cross-sectional study.

Objectives: Fontan-associated liver disease (FALD) is a hallmark of the failing Fontan circulation, but no general classification of FALD severity exists. In this study, we propose a scoring system to grade the severity of FALD and analyse its applicability for evaluation of Fontan failure.

Methods: From 2017 to 2019, a total of 129 successive Fontan patients received a comprehensive hepatic assessment.

Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics.

Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest, n = 101), liver ultrasound (n = 117) and transient elastography (FibroScan, n = 61).