Surveillance of Fontan-associated Liver Disease: Current Standards and a Proposal from the European Society of Paediatric Radiology Abdominal Task Force

Overview

Journal Pediatr Radiol

Specialty Pediatrics

Date 2021 Oct 16

PMID 34654967

Citations 5

Authors

Giulia Perucca

Charlotte de Lange

Stephanie Franchi-Abella

Marcello Napolitano

Michael Riccabona

Damjana Kljucevsek

Seema Toso

Jochen Herrmann

Samuel Stafrace

Kassa Darge

Maria Beatrice Damasio

Costanza Bruno

Magdalena Maria Wozniak

Luisa Lobo

Donald Ibe

Anne M Smets

Philippe Petit

Lil-Sofie Ording Muller

Affiliations

Soon will be listed here.

Abstract

Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

Citing Articles

Multiple Liver Nodules in Fontan-Associated Liver Disease.

Mendes R, Marques L, Figueiredo P GE Port J Gastroenterol. 2024; 31(1):70-72.

PMID: 38476301 PMC: 10928859. DOI: 10.1159/000528462.

Liver Biopsy Leads to Serendipitous Diagnosis of Glycogen Storage Disease Type IX in a Patient With Fontan-Associated Liver Disease.

Hyer E, Alexander C, Rand E, Diamond T JPGN Rep. 2023; 4(4):e377.

PMID: 38034453 PMC: 10684236. DOI: 10.1097/PG9.0000000000000377.

Fontan-associated liver disease: Diagnosis, surveillance, and management.

de Lange C, Moller T, Hebelka H Front Pediatr. 2023; 11:1100514.

PMID: 36937979 PMC: 10020358. DOI: 10.3389/fped.2023.1100514.

Assessment of liver fibrosis using a 3-dimensional high-resolution late gadolinium enhancement sequence in children and adolescents with Fontan circulation.

Acosta Izquierdo L, Rai A, Saprungruang A, Chavhan G, Kamath B, Dipchand A Eur Radiol. 2023; 33(8):5446-5454.

PMID: 36786904 DOI: 10.1007/s00330-023-09452-z.

Liver cirrhosis in children - the role of imaging in the diagnostic pathway.

Herrmann J, Petit P, Grabhorn E, Lenz A, Jurgens J, Franchi-Albella S Pediatr Radiol. 2022; 53(4):714-726.

PMID: 36040526 PMC: 10027649. DOI: 10.1007/s00247-022-05480-x.

References

Komatsu H, Inui A, Kishiki K, Kawai H, Yoshio S, Osawa Y . Liver disease secondary to congenital heart disease in children. Expert Rev Gastroenterol Hepatol. 2019; 13(7):651-666. DOI: 10.1080/17474124.2019.1621746. View

Louie C, Pham M, Daugherty T, Kambham N, Higgins J . The liver in heart failure: a biopsy and explant series of the histopathologic and laboratory findings with a particular focus on pre-cardiac transplant evaluation. Mod Pathol. 2015; 28(7):932-43. DOI: 10.1038/modpathol.2015.40. View

Mjelle A, Mulabecirovic A, Havre R, Rosendahl K, Juliusson P, Olafsdottir E . Normal Liver Stiffness Values in Children: A Comparison of Three Different Elastography Methods. J Pediatr Gastroenterol Nutr. 2019; 68(5):706-712. PMC: 6510324. DOI: 10.1097/MPG.0000000000002320. View

Wells M, Hough D, Fidler J, Kamath P, Poterucha J, Venkatesh S . Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma. Abdom Radiol (NY). 2017; 42(11):2623-2631. DOI: 10.1007/s00261-017-1181-9. View

Goldberg D, Surrey L, Glatz A, Dodds K, OByrne M, Lin H . Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study. J Am Heart Assoc. 2017; 6(5). PMC: 5524062. DOI: 10.1161/JAHA.116.004809. View

Tellez L, Rodriguez de Santiago E, Minguez B, Payance A, Clemente A, Baiges A . Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort. J Hepatol. 2019; 72(4):702-710. DOI: 10.1016/j.jhep.2019.10.027. View

Bryant T, Ahmad Z, Millward-Sadler H, Burney K, Stedman B, Kendall T . Arterialised hepatic nodules in the Fontan circulation: hepatico-cardiac interactions. Int J Cardiol. 2010; 151(3):268-72. DOI: 10.1016/j.ijcard.2010.05.047. View

Chavhan G, Shelmerdine S, Jhaveri K, Babyn P . Liver MR Imaging in Children: Current Concepts and Technique. Radiographics. 2016; 36(5):1517-32. DOI: 10.1148/rg.2016160017. View

Guimaraes A, Siqueira L, Uppal R, Alford J, Fuchs B, Yamada S . T2 relaxation time is related to liver fibrosis severity. Quant Imaging Med Surg. 2016; 6(2):103-14. PMC: 4858462. DOI: 10.21037/qims.2016.03.02. View

10.

Parker J, Weller M, Feinstein L, Adams R, Main M, Grayburn P . Safety of ultrasound contrast agents in patients with known or suspected cardiac shunts. Am J Cardiol. 2013; 112(7):1039-45. DOI: 10.1016/j.amjcard.2013.05.042. View

11.

Daniels C, Bradley E, Landzberg M, Aboulhosn J, Beekman 3rd R, Book W . Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol. 2017; 70(25):3173-3194. DOI: 10.1016/j.jacc.2017.10.045. View

12.

Li X, Li C, Wang R, Ren J, Yang J, Zhang Y . Combined Application of Gadoxetic Acid Disodium-Enhanced Magnetic Resonance Imaging (MRI) and Diffusion-Weighted Imaging (DWI) in the Diagnosis of Chronic Liver Disease-Induced Hepatocellular Carcinoma: A Meta-Analysis. PLoS One. 2015; 10(12):e0144247. PMC: 4668097. DOI: 10.1371/journal.pone.0144247. View

13.

Oh C, Youn J, Han J, Kim G, Kim H, Jung S . Hepatocellular carcinoma after the Fontan procedure in a 16-year-old girl: A case report. Medicine (Baltimore). 2016; 95(41):e4823. PMC: 5072929. DOI: 10.1097/MD.0000000000004823. View

14.

Silva-Sepulveda J, Fonseca Y, Vodkin I, Vaughn G, Newbury R, Vavinskaya V . Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics. Congenit Heart Dis. 2019; 14(4):600-608. DOI: 10.1111/chd.12770. View

15.

Dillman J, Trout A, Alsaied T, Gupta A, Lubert A . Imaging of Fontan-associated liver disease. Pediatr Radiol. 2020; 50(11):1528-1541. DOI: 10.1007/s00247-020-04776-0. View

16.

Behairy B, Sira M, Zalata K, Salama E, Abd-Allah M . Transient elastography compared to liver biopsy and morphometry for predicting fibrosis in pediatric chronic liver disease: Does etiology matter?. World J Gastroenterol. 2016; 22(16):4238-49. PMC: 4837441. DOI: 10.3748/wjg.v22.i16.4238. View

17.

Shiina T, Nightingale K, Palmeri M, Hall T, Bamber J, Barr R . WFUMB guidelines and recommendations for clinical use of ultrasound elastography: Part 1: basic principles and terminology. Ultrasound Med Biol. 2015; 41(5):1126-47. DOI: 10.1016/j.ultrasmedbio.2015.03.009. View

18.

Stellin G . A Tribute to the Pioneers of Right Heart Bypass: An Historical Review. World J Pediatr Congenit Heart Surg. 2020; 11(2):198-203. DOI: 10.1177/2150135119894478. View

19.

Nievelstein R, van Dam I, van der Molen A . Multidetector CT in children: current concepts and dose reduction strategies. Pediatr Radiol. 2010; 40(8):1324-44. PMC: 2895901. DOI: 10.1007/s00247-010-1714-7. View

20.

Munsterman I, Duijnhouwer A, Kendall T, Bronkhorst C, Ronot M, Wettere M . The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort. Eur Heart J. 2018; 40(13):1057-1068. DOI: 10.1093/eurheartj/ehy620. View