Hepatocellular carcinoma after the Fontan procedure in a 16-year-old girl

Abstract

Introduction:The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC).Clinical Findings:We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue. Imaging studies revealed a large hepatic mass involving most of the right lobe and multiple masses in the left lobe. Evidence suggested severe liver cirrhosis, and the presence of ascites, hepatosplenomegaly, paraesophageal gastric varices reflecting the severity of the disease. In addition, tumor thrombosis was found in the right hepatic vein, middle hepatic vein, and inferior vena cava, as well as multiple metastatic nodules in both lungs. The patient received an incisional biopsy and the diagnosis of HCC was pathologically confirmed. After treatment with 1 cycle of systemic chemotherapy, she received ongoing supportive care for disease-related complications, and died 2 months after chemotherapy due to hematemesis.Conclusion:With the advances in medicine, the incidence of Fontan physiology-related complications is likely to increase, and the incidence of HCC will also increase accordingly. As early diagnosis of HCC results in better patient outcomes, a surveillance guideline for HCC after the FP should be developed.

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

“…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.…”

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

“…Cardiopulmonary exercise testing provides such information including peak oxygen consumption (VO 2 ), heart rate reserve, and VE/VCO 2 slope, a marker of respiratory efficiency. [48][49][50] Ideally, a liver transplant is the first approach to adult patients with cirrhosis who develop HCC. Literature on the topic was reviewed, including reports that a peak VO 2 < 16.6 mL/kg/min and a peak heart rate <122 bpm have been predictive of mortality, whereas VO 2 at anaerobic threshold <10 mL/kg/min and VE/VCO 2 slope >35 have been associated with hospitalization, protein-losing enteropathy, and heart failure in younger patients.…”

“…[44][45][46][47] What to do with patients who develop hepatocellular carcinoma is another dilemma. [48][49][50] Ideally, a liver transplant is the first approach to adult patients with cirrhosis who develop HCC. 14,51,52 In the Fontan population, a liver transplant alone is not an option, and a combined transplant has its own risks to consider.…”

Congestive hepatopathy: Differentiating congestion from fibrosis

“…Supporting evidence for this theory is that a majority of patients with chronic liver congestion have nodular‐appearing livers on imaging, but many of these patients who undergo heart transplantation have no lasting signs of liver dysfunction posttransplant . In addition, there is ongoing debate regarding the management of arterially enhancing hepatic nodules, because it remains unclear whether the standard radiographic guidelines for distinguishing FNH from HCC apply in congestive liver disease . Therefore, guided biopsies of suspicious lesions may still play a role in confirming the diagnosis of HCC in patients with congestive hepatopathy, at least until further studies confirm or refute that radiographic diagnostic criteria are sufficient in these patients.…”

“…Congestive Hepatopathy Lemmer, VanWagner, and Ganger the standard radiographic guidelines for distinguishing FNH from HCC apply in congestive liver disease. 21 Therefore, guided biopsies of suspicious lesions may still play a role in confirming the diagnosis of HCC in patients with congestive hepatopathy, at least until further studies confirm or refute that radiographic diagnostic criteria are sufficient in these patients. Liver stiffness assessments using transient elastography, acoustic radiation force impulse elastography, or magnetic resonance elastography (MRE) all uniformly demonstrate elevated values in congestive hepatopathy because of increased blood volumes within the liver and presinusoidal edema.…”