Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics

• DOI: 10.1007/s00246-020-02291-5
• Publication Date: 2020-02-01T03:02:37Z
• Authors: Anastasia Schleiger; Madeleine Salzmann; Peter Kramer; Friederike Danne; Stephan Schubert; Christian Bassir; Tobias Müller; Hans-Peter Müller; Felix Berger; Stanislav Ovroutski
• Keywords: Adult; Liver Cirrhosis; Male; Cardiac Catheterization; Hemodynamics; Fontan Procedure; Severity of Illness Index; Female [MeSH] ; Elasticity Imaging Techniques [MeSH] ; Risk factors for FALD ; Adult [MeSH] ; Ultrasonography [MeSH] ; Fontan surveillance ; Humans [MeSH] ; Liver Cirrhosis/diagnostic imaging [MeSH] ; Prospective Studies [MeSH] ; Severity of Illness Index [MeSH] ; Risk Factors [MeSH] ; Cross-Sectional Studies [MeSH] ; Original Article ; Liver Cirrhosis/pathology [MeSH] ; Cardiac Catheterization/adverse effects [MeSH] ; Liver Cirrhosis/etiology [MeSH] ; Male [MeSH] ; Fontan Procedure/adverse effects [MeSH] ; Fontan hemodynamics ; Echocardiography [MeSH] ; Fontan-associated liver disease ; Hemodynamics [MeSH] ; Liver cirrhosis ; Child, Preschool [MeSH]; 3. Good health; 03 medical and health sciences; Cross-Sectional Studies; 0302 clinical medicine; Echocardiography; Risk Factors; Child, Preschool; Elasticity Imaging Techniques; Humans; Original Article; Female; Prospective Studies; Ultrasonography

AbstractFontan-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). The median patient age was 16.0 years [IQR 14.2], and the median duration of the Fontan circulation was 10.3 years [IQR 14.7]. Hemodynamic assessment was performed using echocardiography, cardiopulmonary exercise capacity testing and cardiac catheterization. Liver ultrasound revealed hepatic parenchymal changes in 83 patients (70.9%). Severe liver cirrhosis was detectable in 20 patients (17.1%). Median liver stiffness measured by FibroScan® was 27.7 kPa [IQR 14.5], and the median Fibrotest® score was 0.5 [IQR 0.3], corresponding to fibrosis stage ≥ 2. Liver stiffness values and Fibrotest® scores correlated significantly with Fontan duration (P1 = 0.013, P2 = 0.012). Exercise performance was significantly impaired in patients with severe liver cirrhosis (P = 0.003). Pulmonary artery pressure and end-diastolic pressure were highly elevated in cirrhotic patients (P1 = 0.008, P2 = 0.003). Multivariable risk factor analysis revealed Fontan duration to be a major risk factor for the development of FALD (P < 0.001, OR 0.77, CI 0.68–0.87). In the majority of patients, hepatic abnormalities suggestive of FALD were detectable by liver ultrasound, transient elastography and laboratory analysis. The severity of FALD correlated significantly with Fontan duration and impaired Fontan hemodynamics. A detailed hepatic assessment is indispensable for long-term surveillance of Fontan patients.