EASL-ERN position paper on liver involvement in patients with Fontan-type circulation

Téllez, Luis; Payancé, Audrey; Tjwa, Eric; del Cerro, María Jesús; Idorn, Lars; Ovroutski, Stanislav; De Bruyne, Ruth; Verkade, Henkjan J.; De Rita, Fabrizio; de Lange, Charlotte; Angelini, Annalisa; Paradis, Valérie; Rautou, Pierre Emmanuel; García-Pagán, Juan Carlos

doi:10.1016/j.jhep.2023.07.013

Cited by 13 publications

(1 citation statement)

References 340 publications

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“…Recently published European Association for the Study of the Liver (EASL) guidelines, suggest screening starting 10-years post-Fontan. 11 However, based on our analysis, annual incidence 10-20 years after Fontan is between 0-0.2% and cannot be de nitely recommended.…”

Section: Discussionmentioning

confidence: 78%

A meta-analysis of cumulative incidence of hepatocellular carcinoma after the Fontan operation

Hansen,

Gilroy,

Lindsay

et al. 2024

Preprint

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Background Hepatic complications are increasingly recognized after the Fontan operation. The development of hepatocellular carcinoma (HCC) is associated with high mortality when diagnosed, but its incidence and risk factors are poorly understood. We conducted a systematic review and meta-analysis of the cumulative incidence of HCC after Fontan and associated risk factors. Methods We searched PubMed, CINAHL, and MEDLINE databases for articles reporting the cumulative incidence of HCC after Fontan operation on March 21, 2023. A single-arm random effects meta-analysis was conducted to assess cumulative incidence at 10-, 20-, and 30-years after Fontan. Meta-analysis of the difference of the medians was used to assess the influence of risk factors on the development of HCC. Results Four studies including a total of 1,320 patients reported cumulative incidence. The cumulative incidence of HCC at 10-, 20-, and 30-years after Fontan was 0% (95% CI 0.00-0.01), 2% (0.01–0.06), and 7% (0.03–0.17) respectively. Seven studies including 6,250 patients reported overall incidence of HCC and associated risk factors. At a median 18.4 (IQR 11.9–24.9) years of follow-up, incidence of HCC was 2% (0.01–0.04). Only use of anticoagulation was associated with a lower risk of HCC (RR 0.3, 95% CI 0.1–0.88). Discussion By 30 years after Fontan, cumulative incidence of HCC is high (7%). Risk of HCC development prior to 10-years post-Fontan is low (0%) and HCC screening can be safely deferred in this population. Screening with ultrasound every 6 months starting 20-years post-Fontan is reasonable, however, further research regarding timing, cost-effectiveness, additional risk factors associated with HCC risk, and different screening modalities is required.

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Section: Discussionmentioning

confidence: 78%

A meta-analysis of cumulative incidence of hepatocellular carcinoma after the Fontan operation

Hansen,

Gilroy,

Lindsay

et al. 2024

Preprint

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Thrombocytopenia is Associated with Higher Fontan Pressure and Increased Morbidity in Patients with Fontan Circulation

Gould,

Gunsaulus,

Feingold

et al. 2024

Pediatr Cardiol

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Impact of ursodeoxycholic acid treatment on Fontan-associated liver disease

Kogiso,

Ogasawara,

Taniai

et al. 2024

J Gastroenterol

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Background Fontan-associated liver disease (FALD) is a type of progressive liver fibrosis that occurs following Fontan surgery and can be complicated by hepatocellular carcinoma (HCC). Established treatments for FALD are lacking. Therefore, we investigated the efficacy of ursodeoxycholic acid (UDCA) in patients with FALD. Methods This single-center retrospective study was conducted from 2003 to 2024 and involved 220 patients (103 men, 46.8%) who had been diagnosed with FALD. UDCA was administered to 113 patients presenting with liver or biliary enzyme abnormalities. We evaluated the patients’ liver enzyme levels 3, 6, and 12 months after treatment. HCC developed in 10.5% and the mortality rate was 4.5%. Survival and cumulative incidence of HCC were compared between patients with and without UDCA treatment using Kaplan–Meier curves and propensity-matched analysis (n = 68 per group). Results UDCA treatment significantly reduced the aspartate aminotransferase (AST), alanine transaminase (ALT), and gamma-glutamyl transferase (GGT) levels at 3 months. The mean pretreatment AST/ALT/GGT levels were 26/22/323 U/L, respectively, and decreased to 19/15/102 U/L at 3 months, 18/12/88 U/L at 6 months, and 16/19/64 U/L at 12 months. However, the total bilirubin level and platelet count did not show significant differences. The survival rate was higher and the HCC rate was lower in patients with than without UDCA treatment. The 5-year incidence rate of HCC was 5.6% in the UDCA group and 24.2% in the untreated group. Conclusions UDCA treatment significantly reduced liver enzyme levels, including GGT, and mitigated the progression of HCC. UDCA may be beneficial for patients with FALD.

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EASL-ERN position paper on liver involvement in patients with Fontan-type circulation

Cited by 13 publications

References 340 publications

A meta-analysis of cumulative incidence of hepatocellular carcinoma after the Fontan operation

A meta-analysis of cumulative incidence of hepatocellular carcinoma after the Fontan operation

Thrombocytopenia is Associated with Higher Fontan Pressure and Increased Morbidity in Patients with Fontan Circulation

Impact of ursodeoxycholic acid treatment on Fontan-associated liver disease

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