Pediatric Cardiology

, Volume 39, Issue 8, pp 1604–1613 | Cite as

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

  • T. AckermanEmail author
  • A. Geerts
  • H. Van Vlierberghe
  • J. De Backer
  • K. François
Original Article


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 scores in post-Fontan screening remains ambiguous. The high percentage of morphologic liver changes in palliated patients supports the use of US in periodic follow-up. LS likely overestimates fibrosis due to liver congestion, arguing for the need of validation through sequential measurements. Screening should minimally encompass US assessment in combination with selective liver fibrosis scores. The role of LS measurement in Fontan follow-up and liver screening needs to be further elucidated.


Univentricular heart Fontan circulation Cardiac hepatopathy Serum markers Elastography Screening 





Alanine aminotransferase


AST-to-platelet ratio index


Aspartate aminotransferase


Chronic right heart failure


Central venous pressure


Fibrosis 4


Focal nodular hyperplasia




Gamma-glutamyl transferase


Hepatic artery


Hepatocellular carcinoma


Hepatic vein


Inferior caval vein


Interquartile range


Liver stiffness


Magnetic resonance imaging


New York Heart Association


Pulsatility index


Portal vein


Resistance index


Superior mesenteric artery


Shear wave elastography


Total cavopulmonary connection




Univentricular heart


Compliance with Ethical Standards

Conflict of interest

All authors declare that he/she has no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Cardiac SurgeryUniversity Hospital GhentGhentBelgium
  2. 2.Department of HepatologyUniversity Hospital GhentGhentBelgium
  3. 3.Department of CardiologyUniversity Hospital GhentGhentBelgium

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