Hepatic Changes in the Fontan Circulation: Identification of Liver Dysfunction and an Attempt to Streamline Follow-up Screening

Pediatric Cardiology
T AckermanK François

Abstract

We tried to identify structural and functional liver aberrances in a palliated Fontan population and sought to determine useful screening modalities, in order to propose a screening protocol to detect patients at risk. Twenty nine patients, median age 23.7 years (interquartile range (IQR) 20.5-27.2) and median Fontan interval 19.7 years (IQR 4.5-21.4), were prospectively studied with echocardiography, blood analysis (including serum fibrosis scores Forns, APRI and FIB4), liver imaging (ultrasound (US), Doppler), and shear wave elastography to determine liver stiffness (LS). Laboratory tests predominantly showed abnormal values for gamma-glutamyltransferase. Forns index indicated moderate fibrosis in 29% of patients and correlated with Fontan interval (p = 0.034). US liver morphology was deviant in 46% of patients, with surface nodularity in 21% and nodular hyperplasia in 29%. Doppler assessment of flow velocities was within normal ranges for most patients. LS (mean 10.4 ± 3.7 kPa) was elevated in 96% of our population and higher LS values were significantly related to longer Fontan interval (p = 0.018). Adolescent and adult Fontan patients show moderate signs of liver dysfunction. Usefulness of serum parameters and fibrosis sco...Continue Reading

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Citations

May 28, 2019·Expert Review of Gastroenterology & Hepatology·Haruki KomatsuTomoo Fujisawa
May 15, 2020·Cardiology in the Young·Renate KaulitzMichael Hofbeck
Jan 17, 2021·Radiología·M J Parada BlázquezB Vargas Serrano
Jul 15, 2020·Ultrasonography·İlhan HekimsoySüha Süreyya Özbek
Jul 8, 2021·European Journal of Pediatrics·Ruth De BruyneKatrien François

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