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Hepatic changes in the fontan circulation : identification of liver dysfunction and an attempt to streamline follow-up screening

Thomas Ackerman (UGent) , Anja Geerts (UGent) , Hans Van Vlierberghe (UGent) , Julie De Backer (UGent) and Kato François (UGent)
(2018) PEDIATRIC CARDIOLOGY. 39(8). p.1604-1613
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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 23.7years (interquartile range (IQR) 20.5-27.2) and median Fontan interval 19.7years (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 +/- 3.7kPa) 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.
Keywords
Univentricular heart, Fontan circulation, Cardiac hepatopathy, Serum markers, Elastography, Screening, SIMPLE NONINVASIVE INDEX, COAGULATION PROFILE, LONG-TERM, SIGNIFICANT FIBROSIS, FAILING FONTAN, HEART-DISEASE, OPERATION, PATHOLOGY, CIRRHOSIS, PREDICT

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Chicago
Ackerman, Thomas, Anja Geerts, Hans Van Vlierberghe, Julie De Backer, and Kato François. 2018. “Hepatic Changes in the Fontan Circulation : Identification of Liver Dysfunction and an Attempt to Streamline Follow-up Screening.” Pediatric Cardiology 39 (8): 1604–1613.
APA
Ackerman, T., Geerts, A., Van Vlierberghe, H., De Backer, J., & François, K. (2018). Hepatic changes in the fontan circulation : identification of liver dysfunction and an attempt to streamline follow-up screening. PEDIATRIC CARDIOLOGY, 39(8), 1604–1613.
Vancouver
1.
Ackerman T, Geerts A, Van Vlierberghe H, De Backer J, François K. Hepatic changes in the fontan circulation : identification of liver dysfunction and an attempt to streamline follow-up screening. PEDIATRIC CARDIOLOGY. 2018;39(8):1604–13.
MLA
Ackerman, Thomas, Anja Geerts, Hans Van Vlierberghe, et al. “Hepatic Changes in the Fontan Circulation : Identification of Liver Dysfunction and an Attempt to Streamline Follow-up Screening.” PEDIATRIC CARDIOLOGY 39.8 (2018): 1604–1613. Print.
@article{8572575,
  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 23.7years (interquartile range (IQR) 20.5-27.2) and median Fontan interval 19.7years (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 +/- 3.7kPa) 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.},
  author       = {Ackerman, Thomas and Geerts, Anja and Van Vlierberghe, Hans and De Backer, Julie and Fran\c{c}ois, Kato},
  issn         = {0172-0643},
  journal      = {PEDIATRIC CARDIOLOGY},
  keyword      = {Univentricular heart,Fontan circulation,Cardiac hepatopathy,Serum markers,Elastography,Screening,SIMPLE NONINVASIVE INDEX,COAGULATION PROFILE,LONG-TERM,SIGNIFICANT FIBROSIS,FAILING FONTAN,HEART-DISEASE,OPERATION,PATHOLOGY,CIRRHOSIS,PREDICT},
  language     = {eng},
  number       = {8},
  pages        = {1604--1613},
  title        = {Hepatic changes in the fontan circulation : identification of liver dysfunction and an attempt to streamline follow-up screening},
  url          = {http://dx.doi.org/10.1007/s00246-018-1937-1},
  volume       = {39},
  year         = {2018},
}

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