Incidence, Predictors, and Mortality in Patients With Liver Cancer After Fontan Operation

Ohuchi, Hideo; Hayama, Yohsuke; Nakajima, Kimiko; Kurosaki, Kenichi; Shiraishi, Isao; Nakai, Michikazu

doi:10.1161/jaha.120.016617

Cited by 21 publications

(16 citation statements)

References 28 publications

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“…After data collation, four studies were excluded to prevent duplication of data 22–25 . Of the remaining 23 studies, 12 and 13 studies were included in the cirrhosis and HCC cohorts, respectively, with two studies, Wu 2017 and Munsterman 2019, common to both cohorts 4,6–8,26–44 . The included studies in cirrhosis and HCC cohorts are reported in Tables 1 and 2, respectively.…”

Section: Resultsmentioning

confidence: 99%

“…5 The risk of progression of FALD to cirrhosis and subsequent risk of hepatocellular carcinoma (HCC) increases over time, regardless of type of Fontan surgery or age at Fontan. [6][7][8][9] Studies across the globe have reported variable rates of cirrhosis and HCC development, which may be secondary to multiple factors related to patient characteristics and underlying comorbidities. Given the significant morbidity and mortality associated with cirrhosis and HCC, several institutes have adopted their own protocol of routinely screening Fontan patients, often based on past experiences with other chronic liver diseases such as hepatitis C (HCV).…”

Section: Introductionmentioning

confidence: 99%

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Meta‐analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation

Hitawala,

Gopalakrishna,

Mironova

et al. 2024

Aliment Pharmacol Ther

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SummaryBackground and AimsThe Fontan palliation is the final stage of surgery for many children born with univentricular physiology. Almost all Fontan patients develop liver fibrosis which may eventually lead to cirrhosis and hepatocellular carcinoma (HCC). These are important causes of morbidity and mortality in these patients. We performed a systematic review and meta‐analysis to assess the incidence of cirrhosis and HCC in Fontan patients and stratify it based on time since surgery.MethodsA literature search of seven databases identified 1158 records. Studies reporting the number of cirrhosis and HCC cases in Fontan patients and time since Fontan surgery were included. In the cirrhosis cohort, we included only those studies where all patients underwent liver biopsy.ResultsA total of 23 studies were included: 12 and 13 studies in the cirrhosis and HCC cohorts, respectively, with two studies included in both cohorts. The incidence of cirrhosis was 0.97 per 100 patient‐years (95% CI 0.57–1.63), with the incidence and cumulative incidence ≥20 years post Fontan surgery being 1.61 per 100 patient‐years (95% CI 1.24–2.08) and 32.2% (95% CI 25.8%–39.4%), respectively. The incidence of HCC was 0.12 per 100 patient‐years (95% CI 0.07–0.21), with the incidence and cumulative incidence ≥20 years post Fontan surgery being 0.20 per 100 patient‐years (95% CI 0.12–0.35) and 3.9% (95% CI 2.2%–6.8%), respectively. Only about 70% of patients with HCC (20/28) had underlying cirrhosis.ConclusionThe incidence of cirrhosis and HCC increases over time, especially at ≥20 years post Fontan surgery. Studies are needed to further identify at‐risk patients in order to streamline surveillance for these highly morbid conditions.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Meta‐analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation

Hitawala,

Gopalakrishna,

Mironova

et al. 2024

Aliment Pharmacol Ther

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“…The proposed pathophysiology is related to the dual blood supply of the liver, with the hepatic vein in direct continuity with the pulmonary artery, leading to increased susceptibility of hepatic parenchymal injury from decreased portal vein oxygenation and increased intrahepatic congestion, ultimately leading to ischaemic hepatitis, characterised by centrilobular necrosis around the hepatic veins. With each injury cycle, increased fibrosis and cirrhosis occurs, leading to FALD presentation 2–5 15…”

Section: Discussionmentioning

confidence: 99%

“…Elevated central venous pressure and diminished cardiac output lead to sinusoidal dilation, edema and centrilobular hepatocyte hypoxia. These changes lead to hepatic fibrosis, a common feature found in Fontan patients 3–10. As in non-FALD patients, these changes ultimately may lead to cirrhosis and hepatocellular carcinoma (HCC).…”

Section: Introductionmentioning

confidence: 99%

Combined hepatocellular carcinoma-cholangiocarcinoma in a patient with Fontan-associated liver disease

Jou

Kaldas

2022

BMJ Case Rep

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We present a rare case of combined hepatocellular carcinoma-cholangiocarcinoma in a woman with a history of univentricular congenital heart disease requiring multiple corrective operations including Fontan procedure. During workup for elevated alpha fetal protein, a right hepatic lobe lesion was identified with biopsy showing poorly differentiated hepatocellular carcinoma. She underwent successful segment 5 liver resection. Final pathology demonstrated combined hepatocellular carcinoma-cholangiocarcinoma. She was treated with gemcitabine/oxaliplatin adjuvant chemotherapy and had no evidence of recurrent disease at her 12-month follow-up. To our knowledge, this is the first case reported in of successful treatment of this rare malignancy in the setting of Fontan-associated liver disease and highlights the importance of a robust screening protocol in this patient population. Semiannual screening for the development of primary liver malignancy should start by 10 years post-Fontan and continue until heart–liver transplantation may be performed. It is important to note that cirrhosis is not a pre-requisite for the development of hepatocellular carcinoma or cholangiocarcinoma in these patients.

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“…12 In addition, Ohuchi H, et al studied in 339 consecutive Fontan patients demonstrated that elevated APRI and FIB-4 scores together with older age and high plasma levels of AFP could be useful predictors of HCC in patients following Fontan operation. 13 Moreover, Egbe AC, et al reported that patients with HCC had a longer duration of post-Fontan operation along with high serum AFP levels. 5 The MRI with hepatocyte-specific HCC should be performed when the HCC feature was detected by CT scan, including enhancement during the arterial phase and wash out during portal venous phase together with elevated AFP level.…”

Section: Discussionmentioning

confidence: 99%

Liver nodules after the Fontan operation: role of the computerised tomography scan

et al. 2021

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Currently, there is an increasing prevalence of liver nodules in patients following the Fontan operation. The appropriate non-invasive modalities have been applied to assess a diagnosis of Fontan-associated liver disease. The aims of this study were to determine the prevalence and associated factors for the presence of liver nodules using CT scan. A cross-sectional study of 34 patients older than 15 years of age was recruited. Ultrasound upper abdomen, ultrasound liver elastography, and CT scan of the upper abdomen were performed after the Fontan operation. The median age of patients was 20 years (range 14–36 years). The median age at the Fontan operation was 7 years (range 5–17 years) and the duration after the Fontan operation was 12 years (range 4–22 years). The prevalence of liver nodules was 62% as detected by CT scans. Hepatic vein pressure in patients with liver nodules was significantly higher than in those without liver nodules. Hepatic vein pressure above 13 mmHg was a factor associated with liver nodules. There was little agreement between the ultrasound of the upper abdomen and CT scan of the upper abdomen in the evaluation of liver nodules. Hepatic pressure was the only associated factor for the occurrence of liver nodules in patients following the Fontan operation. The prevalence of liver nodules was very high after the Fontan operation. The upper abdomen CT scan should be performed for the surveillance of liver nodules in every Fontan patient over the age of 15 years.

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Incidence, Predictors, and Mortality in Patients With Liver Cancer After Fontan Operation

Cited by 21 publications

References 28 publications

Meta‐analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation

Meta‐analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation

Combined hepatocellular carcinoma-cholangiocarcinoma in a patient with Fontan-associated liver disease

Liver nodules after the Fontan operation: role of the computerised tomography scan

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