A liver mass post-Fontan operation

Rajoriya, Neil; Clift, Paul; Thorne, Sara; Hirschfield, Gideon M.; Ferguson, Jack

doi:10.1093/qjmed/hcu020

Cited by 25 publications

(10 citation statements)

References 6 publications

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“…Twenty-six studies including 65 patients with biopsy-proven HCC were selected for our main analysis: one prospective cohort, 12 six retrospective cohorts 5,6,9,[22][23][24] , five retrospective case series, 7,25-28 and 14 case reports. 10,18,[29][30][31][32][33][34][35][36][37][38][39][40] Eleven reports were from North America, seven from Asia, six from Europe, one from Australia, one from Turkey, and one from both North America and Europe (see Table S1). Four were multicenter studies.…”

Section: Study Characteristicsmentioning

confidence: 99%

Hepatocellular carcinoma after Fontan surgery: A systematic review

Santiago

Téllez

Guerrero

et al. 2020

Hepatology Research

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Aim: Fontan surgery is often the procedure of choice for patients with congenital single effective ventricle. In the long term, elevated systemic venous pressure and chronic ischemia following this procedure could lead to advanced chronic liver disease and there is also a risk of hepatocellular carcinoma (HCC). This review systematically summarizes the characteristics and outcomes of this rare condition. Methods: PubMed and Embase databases were searched from inception to January 2020 for studies reporting on HCC after Fontan surgery. The factors analyzed were clinical presentation, histology, imaging findings, treatments, and survival. Our primary analysis was based on biopsy-proven HCC. Results: The records selected were 26 observational studies (19 case reports/case series and seven cohort studies) including 65 biopsy-proven HCC. Age at the time of HCC diagnosis ranged from 12 to 52 years, and 62% of the patients were female. Only one case occurred earlier than 10 years after Fontan surgery. Twenty patients had no imaging or histological evidence of liver cirrhosis and 78.3% had elevated α-fetoprotein levels. Advanced stage was the most common at diagnosis. The most frequent treatments were transarterial chemoembolization (n = 18) and surgery (n = 12). One-year survival was 50% and only four patients (6.2%) were under liver imaging surveillance. We also analyzed 17 patients with non-biopsy-proven HCC. Conclusions: After Fontan surgery, HCC usually occurs at least 10 years later and can develop in the absence of cirrhosis. Biopsy is mandatory to confirm the diagnosis. Patients were diagnosed at a late stage and survival outcomes were poor, highlighting a need for liver surveillance.

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Section: Study Characteristicsmentioning

confidence: 99%

Hepatocellular carcinoma after Fontan surgery: A systematic review

Santiago

Téllez

Guerrero

et al. 2020

Hepatology Research

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“…A PubMed interface search revealed that as of February 2019, at least 33 patients had been diagnosed with HCC after Fontan palliation (Table 2) [5][6][7][8][9][10][11][12][14][15][16][17][18][19][20][21]. Although this number is small, gender seems not to be associated with HCC.…”

Section: Liver Tumors Associated With Congenital Heart Diseasementioning

confidence: 99%

“…However, since the first report of HCC following Fontan palliation surgery was published in 2005 [5], the reported incidence of HCC in patients who have undergone Fontan palliation has increased [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]. Fontan palliative surgery was introduced in 1968 for patients with congenital heart disease; this surgery separates the systemic and pulmonary circulations, producing a single-ventricle physiology [26].…”

Section: Introductionmentioning

confidence: 99%

Liver disease secondary to congenital heart disease in children

Komatsu

Inui

Kishiki

et al. 2019

Expert Review of Gastroenterology & Hepatology

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Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation. Area covered: The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed. Expert opinion: Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.

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“…The mean (range) age at first diagnosis of LC and HCC was 23 (13-34) and 31 (22-44) years, respectively. The mean (range) age at the first Fontan procedure was 8.2 (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15) years. The mean (range) period from the first Fontan procedure until the diagnosis of LC and/or HCC was 14.5 (5-24) and 21.6 (7-31) years, respectively.…”

Section: The 2nd Surveymentioning

confidence: 99%

Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure　― A Nationwide Survey in Japan ―

Kuwabara

Niwa

Toyoda

et al. 2018

Circ J

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Background: Fontan-associated liver disease (FALD) is an important late complication involving liver dysfunction, such as liver cirrhosis (LC) and hepatocellular carcinoma (HCC), in patients undergoing the Fontan procedure. However, the prevalence, clinical manifestation, and methods of diagnosis of FALD are still not well established. Methods and Results:This study comprised 2 nationwide surveys in Japan. First, the prevalence of LC and/or HCC in patients undergoing the Fontan procedure was determined. Second, clinical manifestations in patients with LC and/or HCC were analyzed, along with data from blood tests, echocardiography, and right heart catheterization. In the 1st survey, of the 2,700 patients who underwent the Fontan procedure, 31 were diagnosed with LC and/or HCC (1.15%), and 5 died due to liver diseases (mortality: 0.19%). In the 2nd survey, data were collected from 17 patients (12 with LC, 2 with HCC, and 3 with LC+HCC. Of these 17 patients, 5 died (mortality: 29.4%). The mean age at diagnosis of LC and HCC was 23 and 31 years, respectively. Computed tomography followed by ultrasound was most frequently used for diagnosis. Blood tests revealed low platelet counts, increased hemoglobin, aspartate aminotransferase, γ-guanosine triphosphate, and total bilirubin levels, and an elevated international normalized ratio of prothrombin time.Conclusions: LC and/or HCC in patients undergoing the Fontan procedure were not rare late complications and were associated with high mortality rates.

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A liver mass post-Fontan operation

Cited by 25 publications

References 6 publications

Hepatocellular carcinoma after Fontan surgery: A systematic review

Hepatocellular carcinoma after Fontan surgery: A systematic review

Liver disease secondary to congenital heart disease in children

Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure　― A Nationwide Survey in Japan ―

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A liver mass post-Fontan operation

Cited by 25 publications

References 6 publications

Hepatocellular carcinoma after Fontan surgery: A systematic review

Hepatocellular carcinoma after Fontan surgery: A systematic review

Liver disease secondary to congenital heart disease in children

Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure ― A Nationwide Survey in Japan ―

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Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure　― A Nationwide Survey in Japan ―