A survey of national practice patterns of HCC surveillance for patients with Fontan-associated liver disease: A call to action

Matthew R Kappus¹, Dempsey Hughes², Daniel R Ganger², Yuli Kim³, Mustafa Bashir⁴, Julie Bonn⁵, Amir Borhani⁶, David Fetzer⁷, Ryan Fischer⁸, Helena Gabriel⁶, Maha Guindi⁹, Elizabeth Hecht¹⁰, Maarouf Hoteit¹¹, Geetika Khanna¹², Richard Krasuski¹³, Sara Lewis¹⁴, Alisha Mavis¹⁵, Frank Miller¹⁶, Cara Morin¹⁷, Thomas Schiano¹⁸, Gary Schooler¹⁹, Alexandra Shingina²⁰, Claude Sirlin²¹, Amit Singal²², Bachir Taouli²³, Rose Tompkins²⁴, Ali Zaidi²⁵, Lisa VanWagner²⁶, Takeshi Yokoo²²

Affiliations

¹ Division of Gastroenterology and Hepatology, Duke University Health System, Durham, North Carolina, USA.
² Division of Gastroenterology and Hepatology, Northwestern University, Chicago, Illinois, USA.
³ Department of Pediatrics, Division of Cardiology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
⁴ Departments of Radiology and Medicine, Center for Advanced Magnetic Resonance Development, Duke University, Durham, North Carolina, USA.
⁵ Department of Pediatrics, Cincinnati Children's Hospital, Cincinnati, Ohio, USA.
⁶ Department of Radiology, Northwestern University, Chicago, Illinois, USA.
⁷ Department of Radiology, UT Southwestern Medical Center, Dallas, Texas, USA.
⁸ Division of Gastroenterology, Children's Mercy Kansas City, Kansas City, Missouri, USA.
⁹ Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California, USA.
¹⁰ Department of Radiology, Weill Cornell Medicine, New York City, New York, USA.
¹¹ Division of Gastroenterology and Hepatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹² Department of Radiology, Emory University, Atlanta, Georgia, USA.
¹³ Department of Internal Medicine, Division of Cardiology, Duke University Health System, Durham, North Carolina, USA.
¹⁴ Department of Radiology, Mount Sinai Medical Center, Miami Beach, Florida, USA.
¹⁵ Department of Pediatrics, Atrium Health, Charlotte, North Carolina, USA.
¹⁶ Department of Radiology, Northwestern Memorial Hospital, Chicago, Illinois, USA.
¹⁷ Department of Radiology, Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, Ohio, USA.
¹⁸ Recanati/Miller Transplantation Institute, Division of Liver Diseases, Mount Sinai Medical Center, New York City, New York.
¹⁹ Department of Radiology, Cincinnati Children's Hospital, Cincinnati, Ohio, USA.
²⁰ Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.
²¹ Liver Imaging Group, Dept. of Radiology, UC San Diego, La Jolla, California, USA.
²² Department of Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
²³ Department of Diagnostic, Molecular, and Interventional Radiology, BioMedical Engineering and Imaging Institute, Icahn School of Medicine at Mount Sinai, New York City, New York, USA.
²⁴ Department of Internal Medicine, Division of Cardiology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
²⁵ Mount Sinai Adult Congenital Heart Disease Center, Mount Sinai Fuster Heart Hospital, Icahn School of Medicine at Mount Sinai, New York City, New York, USA.
²⁶ Division of Digestive and Liver Diseases, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

PMID: 40192530
DOI: 10.1097/LVT.0000000000000622

A survey of national practice patterns of HCC surveillance for patients with Fontan-associated liver disease: A call to action

Matthew R Kappus et al. Liver Transpl. 2025.

. 2025 Sep 1;31(9):1185-1189.

doi: 10.1097/LVT.0000000000000622. Epub 2025 Apr 8.

Authors

Affiliations

¹ Division of Gastroenterology and Hepatology, Duke University Health System, Durham, North Carolina, USA.
² Division of Gastroenterology and Hepatology, Northwestern University, Chicago, Illinois, USA.
³ Department of Pediatrics, Division of Cardiology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
⁴ Departments of Radiology and Medicine, Center for Advanced Magnetic Resonance Development, Duke University, Durham, North Carolina, USA.
⁵ Department of Pediatrics, Cincinnati Children's Hospital, Cincinnati, Ohio, USA.
⁶ Department of Radiology, Northwestern University, Chicago, Illinois, USA.
⁷ Department of Radiology, UT Southwestern Medical Center, Dallas, Texas, USA.
⁸ Division of Gastroenterology, Children's Mercy Kansas City, Kansas City, Missouri, USA.
⁹ Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California, USA.
¹⁰ Department of Radiology, Weill Cornell Medicine, New York City, New York, USA.
¹¹ Division of Gastroenterology and Hepatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹² Department of Radiology, Emory University, Atlanta, Georgia, USA.
¹³ Department of Internal Medicine, Division of Cardiology, Duke University Health System, Durham, North Carolina, USA.
¹⁴ Department of Radiology, Mount Sinai Medical Center, Miami Beach, Florida, USA.
¹⁵ Department of Pediatrics, Atrium Health, Charlotte, North Carolina, USA.
¹⁶ Department of Radiology, Northwestern Memorial Hospital, Chicago, Illinois, USA.
¹⁷ Department of Radiology, Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, Ohio, USA.
¹⁸ Recanati/Miller Transplantation Institute, Division of Liver Diseases, Mount Sinai Medical Center, New York City, New York.
¹⁹ Department of Radiology, Cincinnati Children's Hospital, Cincinnati, Ohio, USA.
²⁰ Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.
²¹ Liver Imaging Group, Dept. of Radiology, UC San Diego, La Jolla, California, USA.
²² Department of Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
²³ Department of Diagnostic, Molecular, and Interventional Radiology, BioMedical Engineering and Imaging Institute, Icahn School of Medicine at Mount Sinai, New York City, New York, USA.
²⁴ Department of Internal Medicine, Division of Cardiology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
²⁵ Mount Sinai Adult Congenital Heart Disease Center, Mount Sinai Fuster Heart Hospital, Icahn School of Medicine at Mount Sinai, New York City, New York, USA.
²⁶ Division of Digestive and Liver Diseases, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

PMID: 40192530
DOI: 10.1097/LVT.0000000000000622

No abstract available

Keywords: CT; Fontan; Fontan-associated liver disease; HCC; MRI; imaging; screening; surveillance; ultrasound.

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References

1. Agnoletti G, Ferraro G, Bordese R, Marini D, Gala S, Bergamasco L, et al. Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy? Int J Cardiol. 2016;209:60–65.
1. Hitawala AA, Gopalakrishna H, Mironova M, Livinski AA, Wright EC, Downing T, et al. Meta-analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation. Aliment Pharmacol Ther. 2024;59:1020–1032.
1. Di Maria MV, Brown DW, Cetta F, Ginde S, Goldberg D, Menon SC, et al. Surveillance testing and preventive care after Fontan operation: A multi-institutional survey. Pediatr Cardiol. 2019;40:110–115.
1. Emamaullee J, Zaidi AN, Schiano T, Kahn J, Valentino PL, Hofer RE, et al. Fontan-associated liver disease: Screening, management, and transplant considerations. Circulation. 2020;142:591–604.
1. Téllez L, Payancé A, Tjwa E, del Cerro MJ, Idorn L, Ovroutski S, et al. EASL-ERN position paper on liver involvement in patients with Fontan-type circulation. J Hepatol. 2023;79:1270–1301.

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A survey of national practice patterns of HCC surveillance for patients with Fontan-associated liver disease: A call to action

Affiliations

A survey of national practice patterns of HCC surveillance for patients with Fontan-associated liver disease: A call to action

Authors

Affiliations

References

LinkOut - more resources

Full Text Sources

Miscellaneous