TY - JOUR
T1 - Hepatocellular carcinoma and the Fontan circulation
T2 - Clinical presentation and outcomes
AU - Possner, Mathias
AU - Gordon-Walker, Timothy
AU - Egbe, Alexander C.
AU - Poterucha, Joseph T.
AU - Warnes, Carole A.
AU - Connolly, Heidi M.
AU - Ginde, Salil
AU - Clift, Paul
AU - Kogon, Brian
AU - Book, Wendy M.
AU - Walker, Niki
AU - Wagenaar, Lodewijk J.
AU - Moe, Tabitha
AU - Oechslin, Erwin
AU - Kay, W. Aaron
AU - Norris, Mark
AU - Dillman, Jonathan R.
AU - Trout, Andrew T.
AU - Anwar, Nadeem
AU - Hoskoppal, Arvind
AU - Broering, Dieter C.
AU - Bzeizi, Khalid
AU - Veldtman, Gruschen
N1 - Publisher Copyright:
© 2020
PY - 2021/1/1
Y1 - 2021/1/1
N2 - 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.
AB - 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.
KW - Adult congenital heart disease
KW - Alpha-fetoprotein
KW - Fontan
KW - Fontan-associated liver disease
KW - Hepatocellular carcinoma
KW - Liver cirrhosis
UR - http://www.scopus.com/inward/record.url?scp=85089963427&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85089963427&partnerID=8YFLogxK
U2 - 10.1016/j.ijcard.2020.08.057
DO - 10.1016/j.ijcard.2020.08.057
M3 - Article
C2 - 32828959
AN - SCOPUS:85089963427
SN - 0167-5273
VL - 322
SP - 142
EP - 148
JO - International Journal of Cardiology
JF - International Journal of Cardiology
ER -