Hepatocellular Carcinoma after the Fontan Procedure

Asrani, Sumeet K.; Warnes, Carole A.; Kamath, Patrick S.

doi:10.1056/nejmc1214222

Cited by 172 publications

(141 citation statements)

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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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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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“…119,127,[347][348][349] The liver in the Fontan circulation is at risk for FALD (see Liver), which is characterized by congestion, fibrosis, and cirrhosis, which ultimately places these patients at risk for HCC. 120 In addition to being subjected to the physiological alterations characteristic of Fontan physiology (increased central venous pressure and low cardiac output), the liver in the patient with a single ventricle is vulnerable to a number of insults to the liver that begin well before Fontan completion (hypoxia, low output state, and perioperative insult).…”

Section: Special Populationsmentioning

confidence: 99%

“…[127][128][129][130][131] MR imaging with a hepatocyte-specific contrast agent has emerged as the preferred imaging modality for characterization of liver masses but may not be an option in the substantial subgroup of patients with CHD with pacemakers or defibrillators and may be limited in those with coils, vascular plugs, or other metallic devices. 132 Newer contrast-enhanced ultrasonography techniques recently have been approved for use in the United States and may increase the diagnostic accuracy of ultrasound for characterizing focal liver lesions.…”

Section: Managementmentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

180

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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“…(143)(144)(145) Screening for hepatic adenomas by annual liver imaging and alpha-feto protein levels has been recommended and is a low cost way of identifying these cases early in their disease course. (146)(147)(148) …”

Section: Hepatocellular Carcinomamentioning

confidence: 99%

Management of the Fontan circulation in the adult patient

Clift

2017

SAHJ

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Hepatocellular Carcinoma after the Fontan Procedure

Cited by 172 publications

References 5 publications

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

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

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Management of the Fontan circulation in the adult patient

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Hepatocellular Carcinoma after the Fontan Procedure

Cited by 172 publications

References 5 publications

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

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

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Management of the Fontan circulation in the adult patient

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Product

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

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