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

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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Living Donor Liver Transplantation for Advanced Hepatocellular Carcinoma Using â€œNo-touchâ€ Technique: First Case in Myanmar

10.26226/morressier.58eb975ed462b80290b4fe3b ◽

2017 ◽

Author(s):

Tin Tin Mar

Keyword(s):

Hepatocellular Carcinoma ◽

Liver Transplantation ◽

Living Donor ◽

Living Donor Liver Transplantation ◽

Advanced Hepatocellular Carcinoma ◽

Donor Liver ◽

Donor Liver Transplantation ◽

First Case

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Fontan-associated liver disease and hepatocellular carcinoma in adults

Scientific Reports ◽

10.1038/s41598-020-78840-y ◽

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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Transplant-Amenable Hepatocellular Carcinoma in a Fontan Patient

Case Reports in Gastroenterology ◽

10.1159/000501198 ◽

2019 ◽

Vol 13 (2) ◽

pp. 275-279 ◽

Cited By ~ 2

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.

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

SAGE Open Medical Case Reports ◽

10.1177/2050313x20922030 ◽

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.

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Living donor liver transplantation prior to multiple myeloma treatment in a patient with hepatitis B-associated hepatocellular carcinoma and liver cirrhosis: a case report

Annals of Surgical Treatment and Research ◽

10.4174/astr.2018.94.4.216 ◽

2018 ◽

Vol 94 (4) ◽

pp. 216

Author(s):

Chan Woo Cho ◽

Nuri Lee ◽

Gyu-Seong Choi ◽

Jong Man Kim ◽

Choon Hyuck David Kwon ◽

...

Keyword(s):

Hepatocellular Carcinoma ◽

Multiple Myeloma ◽

Liver Transplantation ◽

Liver Cirrhosis ◽

Case Report ◽

Hepatitis B ◽

Living Donor ◽

Living Donor Liver Transplantation ◽

Donor Liver ◽

Donor Liver Transplantation

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

Cancers ◽

10.3390/cancers12071805 ◽

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.

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