scholarly journals Transplant-Amenable Hepatocellular Carcinoma in a Fontan Patient

2019 ◽  
Vol 13 (2) ◽  
pp. 275-279 ◽  
Author(s):  
Brian Patrick Rutledge ◽  
Anupama Devara ◽  
Sindhuri Benjaram ◽  
Stephanie Judd ◽  
Murray Ehrinpreis
Keyword(s):  
Hepatocellular Carcinoma ◽  
At Risk ◽  
Liver Transplant ◽  
Fontan Procedure ◽  
Venous Pressure ◽  
Fontan Patient ◽  
Number Of Patients ◽  
Fontan Physiology ◽  
The Ideal

The Fontan circulation alters a patient’s physiology and imparts long-term risks related to chronically elevated systemic venous pressure. An increasing number of patients with Fontan physiology are surviving into adulthood and are at risk of hepatic sequalae. The ideal timeline and method of hepatic surveillance in the Fontan population remains to be defined. In this case, the patient was diagnosed with hepatocellular carcinoma more than 20 years after undergoing the Fontan procedure and was a candidate for combined heart-liver transplant. That her disease progressed prior to organ availability supports the argument for hepatic surveillance in this population.

scholarly journals Laparoscopic partial liver resection for hepatocellular carcinoma arising from Fontan-associated liver disease: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Miku Iwata ◽  
Katsunori Sakamoto ◽  
Chihiro Ito ◽  
Akimasa Sakamoto ◽  
Mio Uraoka ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Resection ◽  
Central Venous Pressure ◽  
Hepatic Vein ◽  
Fontan Procedure ◽  
Venous Pressure ◽  
Central Venous ◽  
Left Hepatic Vein ◽  
Partial Liver Resection ◽  
Number Of Patients

Abstract Background The Fontan procedure (FP) is a palliative surgery for functional single ventricle. The Fontan circulation maintains pulmonary circulation by a high central venous pressure, leading to chronic congestive liver. The number of patients diagnosed with hepatocellular carcinoma (HCC) arising from liver fibrosis and cirrhosis after FP is increasing. Several reports have described surgical treatment for HCC after FP, but few have described laparoscopic surgery. Case presentation The patient was a 31-year-old man who had undergone the FP for single right ventricle at 3 years. Several liver masses were detected at 30 years. A liver mass in segment 3 showed increasing size concomitant with increasing alpha-fetoprotein concentration, and a solitary HCC 15 mm in diameter was diagnosed. The tumor was located on the liver surface, abutting the origin of the left hepatic vein. Laparoscopic partial liver resection was performed. The postoperative course was uneventful and the patient was discharged on postoperative day 3. The patient remained disease-free on follow-up after 7 months. Conclusions Although we had some concerns, such as difficulty managing general anesthesia and easy venous bleeding due to high central venous pressure, laparoscopic partial liver resection was performed with safe exposure of the left hepatic vein.

scholarly journals Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

2018 ◽  
Vol 38 (6) ◽  
pp. e5-e12 ◽  
Author(s):  
Christine Peyton
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Complex Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Venous Hypertension ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
Fontan Physiology

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Patients with single-ventricle physiology who have undergone the Fontan procedure are at risk for these conditions. Fontan physiology predisposes patients to chronically low cardiac output, increased central venous pressure, and congestive heart failure. These altered hemodynamics lead to increased mesenteric vascular resistance, resulting in venous hypertension and congestion in protein-losing enteropathy. Plastic bronchitis is a complex disease in which chronic high lymphatic pressures from Fontan physiology cause acellular bronchial casts to develop. These entities may also occur in patients with normal Fontan hemodynamics. This article also covers medical and surgical interventions for protein-losing enteropathy and plastic bronchitis. (Critical Care Nurse. 2018;38[6]:e5–e12)

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.

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.

HCC Risk Scores: Useful or Not?

2017 ◽  
Vol 37 (04) ◽  
pp. 287-295 ◽  
Author(s):  
Morris Sherman
Keyword(s):  
Hepatocellular Carcinoma ◽  
At Risk ◽  
Hepatitis B ◽  
External Validation ◽  
Risk Scores ◽  
Before And After ◽  
Changes In Risk ◽  
Long Term Performance ◽  
Term Performance

AbstractThe advent and efficacy of surveillance for hepatocellular carcinoma (HCC) has necessitated the refinement of assessing who is at risk for this cancer. Initially, risk was assessed for all individuals with hepatitis B and all those with cirrhosis. However, the majority of these individuals do not develop HCC so that providing surveillance for all is a waste of resources. There are now many different scores that have been developed that allow better identification of who is at risk and who is not. Specific models have been developed for hepatitis B before and on treatment, for hepatitis C before and after treatment, and for cirrhosis in general. There are also models for assessing risk in the general population. Some models can only be applied to patients coming from the population in which the score was developed (e.g., hepatitis B). Others are more generalizable. Many lack external validation. With some exceptions, the models do not attempt to assess the score at which surveillance should start. Overall, the models provide some useful guidance as to who does not need to undergo surveillance, but the long-term performance and how changes in risk score correlate with changes in HCC risk has not been completely assessed.

scholarly journals Liver transplantation in an adult patient with hepatocellular carcinoma following liver cirrhosis as a complication of the Fontan procedure -A case report-

2020 ◽  
Vol 15 (4) ◽  
pp. 466-471
Author(s):  
Min-kyung Cho ◽  
Ji-Hye Kwon ◽  
Mi Sook Gwak ◽  
Jae-Won Joh ◽  
JiYun Hwang ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Transplantation ◽  
Cardiac Output ◽  
Liver Cirrhosis ◽  
Multidisciplinary Approach ◽  
Fontan Procedure ◽  
Venous Pressure ◽  
Pulmonary Atresia ◽  
Hepatic Disorder ◽  
First Case

Background: Fontan-associated liver disease (FALD) is a hepatic disorder caused by hemodynamic changes and systemic venous congestion following the Fontan procedure. FALD includes liver cirrhosis and hepatocellular carcinoma (HCC), both of which may require liver transplantation (LT). However, the Fontan circulation, characterized by elevated central venous pressure and reduced cardiac output, is a challenging issue for surgeons and anesthesiologists.Case: We report a living-donor LT for the treatment of HCC. The patient was a 24-year-old male who underwent the Fontan procedure for pulmonary atresia and right ventricle hypoplasia. We focused on maintaining enough blood volume for cardiac output without causing pulmonary edema, as the patient is not well adapted to changes in volume. Owing to a multidisciplinary approach, the surgery was successfully performed without fatal adverse events.Conclusions: To our knowledge, this is the first case of isolated LT in a recipient who became an adult after having undergone the Fontan procedure.

Sign in / Sign up

Export Citation Format

Share Document