Mathias Possner1, Timothy Gordon-Walker2, Alexander C Egbe3, Joseph T Poterucha3, Carole A Warnes3, Heidi M Connolly3, Salil Ginde4, Paul Clift5, Brian Kogon6, Wendy M Book7, Niki Walker8, Lodewijk J Wagenaar9, Tabitha Moe10, Erwin Oechslin11, W Aaron Kay12, Mark Norris13, Jonathan R Dillman14, Andrew T Trout14, Nadeem Anwar15, Arvind Hoskoppal16, Dieter C Broering17, Khalid Bzeizi18, Gruschen Veldtman19. 1. Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington, USA. 2. Scottish Liver Transplant Unit, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom; The University of Edinburgh, Edinburgh, United Kingdom. 3. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA. 4. Division of Pediatric Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA. 5. Department of Cardiology, New Queen Elizabeth Hospital, Birmingham, United Kingdom. 6. Department of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Egleston, Emory University, Atlanta, GA, USA. 7. Division of Cardiology, Department of Medicine, Emory University Department of Medicine, Atlanta, GA, USA. 8. Scottish Adult Congenital Cardiac Service, Golden Jubilee National Hospital, Glasgow, United Kingdom. 9. Department of Cardiology, Thoraxcentrum Twente, Medisch Spectrum Twente Hospital, Enschede, the Netherlands. 10. Division of Cardiology, Phoenix Children's Hospital, Phoenix, AZ, USA. 11. Toronto Congenital Cardiac Centre for Adults, University Health Network / Peter Munk Cardiac Centre, and University of Toronto, Toronto, Ontario, Canada. 12. Section of Pediatric Cardiology, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, USA; Krannert Institute of Cardiology, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA. 13. Division of Pediatric Cardiology, University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI, USA. 14. Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. 15. Division of Gastroenterology, Department of Medicine, University of Cincinnati College of Medicine, Cincinnati, OH, USA. 16. Department of Pediatrics, University of Utah and Intermountain Healthcare, Salt Lake City, UT, USA. 17. Department of Liver Transplantation, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. 18. Adult Congenital Heart Disease, Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. 19. Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Adult Congenital Heart Disease, Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. Electronic address: Gruschen@me.com.
Abstract
BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage. Crown
BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage. Crown
Authors: Václav Chaloupecký; Denisa Jičínská; Viktor Tomek; Ondřej Materna; Roman Gebauer; Rudolf Poruban; Petra Antonová; Theodor Adla; Matěj Štefánek; Vojtěch Illinger; Karel Kotaška; Jan Janoušek Journal: Front Cardiovasc Med Date: 2022-09-29