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.

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)

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 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 Spontaneously ruptured hepatocellular carcinoma in Fontan-associated liver disease: A case report

2020 ◽  
Vol 8 ◽  
pp. 2050313X2092203
Author(s):  
Takahiro Kubo ◽  
Yosuke Aihara ◽  
Hideto Kawaratani ◽  
Tadashi Namisaki ◽  
Ryuichi Noguchi ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Disease ◽  
Transcatheter Arterial Embolization ◽  
Fontan Procedure ◽  
Arterial Embolization ◽  
Palliative Operation ◽  
First Case ◽  
Ruptured Hepatocellular Carcinoma ◽  
Enhanced Computed Tomography

The prognosis of congenital heart disease is dramatically improved by cardiac surgery. The Fontan procedure is the definitive palliative operation for patients with single-ventricle physiology. In parallel with the longer survival time achieved with the Fontan procedure, the incidence of Fontan-associated liver disease is increasing. A 40-year-old man who underwent Fontan procedures at the ages of 9 was referred to our hospital for further evaluation of multiple hepatic tumors. Enhanced computed tomography showed large hepatocellular carcinomas with portal thrombi (Vp3). Spontaneous hepatocellular carcinoma rupture occurred 2 weeks after the first visit to our hospital, and emergent transcatheter arterial embolization of the hepatic artery was performed. Three months later, the patient died of liver failure. Autopsy findings showed moderately differentiated hepatocellular carcinoma with a cirrhotic liver characterized by centrilobular fibrosis and sinusoidal dilation similar to that in Fontan-associated liver disease. We reported the first case of spontaneously ruptured hepatocellular carcinoma treated by emergent transcatheter arterial embolization in Fontan-associated liver disease. As the early diagnosis of liver cirrhosis and hepatocellular carcinoma results in better patients’ outcome, cardiologists and hepatologists should be aware of Fontan-associated liver disease and advise patients to have regular follow-up of the liver.

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.

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.

In Vitro Study of Pulmonary Vascular Resistance in Fontan Circulation With Respiration Effects

2012 ◽  
Author(s):  
Marija Vukicevic ◽  
Timothy A. Conover ◽  
Jian Zhou ◽  
Tain-Yen Hsia ◽  
Richard S. Figliola
Keyword(s):  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Heart Defects ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Pulmonary Arteries ◽  
Pulmonary Regurgitation ◽  
Vena Cava

The Fontan operation is the final stage of palliative surgery for children born with single ventricle heart defects. The most common configuration is called total cavopulmonary connection (TCPC), wherein the inferior vena cava and superior vena cava are anastomosed directly to the pulmonary arteries; therefore the pulmonary circulation is driven by venous pressure only. The Fontan procedure, although successful in the early postoperative period, with time can decrease in efficiency or even fail within several years after the operation. The reasons of different clinical outcomes for some of the Fontan patients are not clear enough, even though it is commonly accepted that certain factors such as low pulmonary vascular resistance and proper shape and size of the TCPC construction are crucial for the succesful long term outcomes. Accordingly, one of the major problems is the increase in pulmonary vascular resistance due to altered hemodynamics after the surgery, causing venous hypertension and respiratory-dependent pulmonary regurgitation [1]. The main pulmonary arteries may also see increased resistance due to congenital malformations, surgical scarring, or deliberate surgical banding. Thus, the consequence of the increased pulmonary vascular resistance at both proximal and distal locations with respect to the TCPC junction, and its effect on the systemic pressures and flow rates, is the main objective of this study.

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.

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