Hepatocellular Carcinoma: A Rare Complication of Cardiac Cirrhosis From Fontan Procedure

2015 ◽  
Vol 110 ◽  
pp. S374
Author(s):  
Tyffany Ping-Yu Chen ◽  
Daniel Shue ◽  
John Daniels ◽  
Brian Kim
Keyword(s):  
Hepatocellular Carcinoma ◽  
Rare Complication ◽  
Fontan Procedure ◽  
Cardiac Cirrhosis

scholarly journals Surgical Resection for Hepatocellular Carcinoma with Cardiac Cirrhosis after the Fontan Procedure

2016 ◽  
Vol 55 (22) ◽  
pp. 3265-3272 ◽  
Author(s):  
Yoshitaka Takuma ◽  
Yuji Fukada ◽  
Shota Iwadou ◽  
Hirokazu Miyatake ◽  
Shuji Uematsu ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Surgical Resection ◽  
Fontan Procedure ◽  
Cardiac Cirrhosis

scholarly journals Risk of Liver Cirrhosis and Hepatocellular Carcinoma after Fontan Operation: A Need for Surveillance

Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1805
Author(s):  
Jun Sik Yoon ◽  
Dong Ho Lee ◽  
Eun Ju Cho ◽  
Mi Kyoung Song ◽  
Young Hun Choi ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Cumulative Incidence ◽  
Rare Complication ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Late Complication ◽  
Elevated Serum ◽  
Arterial Phase ◽  
Portal Venous Phase

Liver cirrhosis and hepatocellular carcinoma (HCC) are serious late complications that can occur after the Fontan procedure. This study aimed to investigate the cumulative incidence of cirrhosis and HCC and to identify specific features distinguishing HCC from benign arterial-phase hyperenhancing (APHE) nodules that developed after the Fontan operation. We retrospectively enrolled 313 post-Fontan patients who had been followed for more than 5 years and had undergone ultrasound or computed tomography (CT) of the liver between January 2000 and August 2018. Cirrhosis was diagnosed radiologically. The estimated cumulative incidence rates of cirrhosis at 5, 10, 20, and 30 years after the Fontan operation were 1.3%, 9.2%, 56.6%, and 97.9%, respectively. Multiphasic CT revealed that 18 patients had APHE nodules that were ≥1 cm in size and showed washout in the portal venous phase (PVP)/delayed phase, which met current noninvasive HCC diagnosis criteria. Among them, only seven patients (38.9%, 7/18) were diagnosed with HCC. After cirrhosis developed, the annual incidence of HCC was 1.04%. The appearance of washout in the PVP (p = 0.006), long time elapsed since the initial Fontan operation (p = 0.04), large nodule size (p = 0.03), and elevated serum α-fetoprotein (AFP) level (p < 0.001) were significantly associated with HCC. In conclusion, cirrhosis is a frequent late complication after Fontan operation, especially after 10 years, and HCC is not a rare complication after cirrhosis development. Diagnosis of HCC should not be based solely on the current imaging criteria, and washout on PVP and clinical features might be helpful to differentiate HCC nodules from benign APHE nodules.

Hepatocellular carcinoma in the adult Fontan patient

2016 ◽  
Vol 27 (2) ◽  
pp. 407-409 ◽  
Author(s):  
Michael R. Conroy ◽  
Tabitha G. Moe
Keyword(s):  
Heart Failure ◽  
Hepatocellular Carcinoma ◽  
Liver Biopsy ◽  
Liver Enzymes ◽  
Fontan Procedure ◽  
Tricuspid Atresia ◽  
Fontan Patient ◽  
Cardiac Cirrhosis ◽  
Fontan Patients ◽  
Carcinoma Hepatocellular

AbstractIn this study, we describe the case of a 36-year-old woman who was diagnosed with hepatocellular carcinoma on a background of Fontan procedure for tricuspid atresia. She had worsening heart failure in the months before presentation, and early investigations noted derangement in liver enzymes and hepatomegaly. Liver biopsy confirmed a hepatocellular carcinoma. Hepatocellular carcinoma is a rare but recognised consequence of cardiac cirrhosis in Fontan patients.

HEPATOCELLULAR CARCINOMA WITH CARDIAC CIRRHOSIS AFTER THE FONTAN PROCEDURE

2018 ◽  
Vol 41 (1) ◽  
pp. 68-71 ◽  
Author(s):  
Nuretdin Suna ◽  
Diğdem Özer Etik ◽  
Serkan Öcal ◽  
Fatih Hilmioğlu
Keyword(s):  
Hepatocellular Carcinoma ◽  
Fontan Procedure ◽  
Cardiac Cirrhosis

scholarly journals An unusual cause of edema in a child with congenital heart disease post Fontan procedure

2019 ◽  
Vol 7 (11) ◽  
pp. 4396
Author(s):  
Minal Wade ◽  
Shweta Shettiwar ◽  
Ankita Shah
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Rare Complication ◽  
Fontan Procedure ◽  
Protein Losing Enteropathy ◽  
Common Causes ◽  
Disease Protein ◽  
The Common ◽  
Fontan Palliation

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

2021 ◽  
Author(s):  
Luis Téllez ◽  
Enrique Rodríguez de Santiago ◽  
Agustín Albillos
Keyword(s):  
Hepatocellular Carcinoma ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Fontan Procedure ◽  
Elevated Risk ◽  
Histological Changes ◽  
Global Management ◽  
Screening Strategies ◽  
History Of ◽  
Fontan Patients

AbstractFontan-associated liver disease is the term used to encompass the disorders arising from abnormal hemodynamic alterations and systemic venous congestion after the Fontan procedure. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is poorly established, many Fontan patients ultimately develop portal hypertension-related complications such as ascites, esophageal varices, malnutrition, and encephalopathy. Fontan survivors also show an elevated risk of hepatocellular carcinoma. Adequate staging of the liver damage is essential to anticipate screening strategies and improve global management.

scholarly journals Fontan-associated liver disease and hepatocellular carcinoma in adults

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Tomomi Kogiso ◽  
Katsutoshi Tokushige
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Palliative Therapy ◽  
Nationwide Survey ◽  
Venous Drainage ◽  
Cardiac Complications ◽  
Sinusoidal Dilatation

AbstractThe Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.

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