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Inicio Cirugía Española (English Edition) Surgical treatment of hepatocarcinoma after Fontan surgery
Información de la revista
Vol. 101. Núm. 10.
Páginas 724-727 (octubre 2023)
Vol. 101. Núm. 10.
Páginas 724-727 (octubre 2023)
Scientific letter
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Surgical treatment of hepatocarcinoma after Fontan surgery
Tratamiento Quirúrgico del Hepatocarcinoma tras Cirugía de Fontan
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37
Elena Payno
Autor para correspondencia
elenapayno@gmail.com

Corresponding author.
, Luz Divina Juez, Jordi Nuñez, Adolfo Lopez Buenadicha, Javier Nuño
Servicio de Cirugía General del Hospital Universitario Ramón y Cajal, Madrid, Spain
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Table 1. Surgical resection in HCC after FP.
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The Fontan procedure (FP) plays a palliative role in complex congenital heart disease with the presence of a functioning single ventricle. FP creates an anastomosis between the systemic venous return and the pulmonary artery to transmit blood to the single ventricular chamber. Currently, and although FP offers a 20-year survival rate over 80%1, hemodynamic changes cause multiple complications as a result of increased systemic venous pressure, which can affect all organs2. Liver disease related to FP is constant and time-dependent; in advanced stages, complications appear as a result of portal hypertension (PHT), hepatocellular carcinoma (HCC) or hepatic encephalopathy.

We present the case of a 32-year-old man diagnosed at birth with a congenital heart malformation, consisting of a tricuspid malformation with right ventricular hypoplasia. FP (bicavopulmonary anastomosis with fenestrated atrial conduit) was performed when he was 3 years of age, with subsequent placement of an epicardial pacemaker due to episodes of atrioventricular block. Some 23 years after the FP, an HCC screening program was started by the Gastroenterology Department with ultrasound scans every 6 months, which showed no suspicious liver nodules initially. After 5 years of follow-up, due to a slight increase in alpha-fetoprotein (from 3.74 IU/mL to 7.20 IU/mL), a multiphase Computerized Tomography (CT) scan was conducted, which identified a 30-mm focal lesion in segment (S) 8 that was not clearly hypervascular, hyperdense in the portal phase and isodense in the equilibrium phase. In addition, another 15 mm subcapsular lesion in was observed in S 7, which was hyperdense in the arterial and portal phases and isodense in the equilibrium phase (Fig. 1).

Figure 1.

Multiphase CT at diagnosis.

(0.46MB).

Given the uncertain diagnosis, needle aspiration of both lesions was requested. The pathological study of the S 8 lesion reported well-differentiated hepatocarcinoma, and the S 7 lesion demonstrated liver cirrhosis with no evidence of malignancy. The case was presented to a multidisciplinary committee: hepato-cardiac transplantation was ruled out due to left pulmonary hypoplasia, and percutaneous treatments were also ruled out due to technical impossibility, so surgical resection was proposed. The extension study was completed with cranial CT and bone scintigraphy, finding no distant disease. A hepatic hemodynamic study was performed, which showed a wedged hepatic venous pressure of 24 mmHg and a free pressure of 23 mmHg, reflecting posthepatic portal hypertension. Elastography showed grade F4 fibrosis, and gastroscopy revealed small esophageal varices. The preoperative MELD index was 13 and corresponded to a Child Pugh stage B7. The surgery was planned and performed in a multidisciplinary manner, with the participation of the General Surgery and Digestive System, Cardiac Surgery, Pediatric Cardiology and Anesthesiology departments. Initially, the Cardiac Surgery unit repositioned the epicardial pacemaker located in the right subcostal region, with intra-surgical verification of the electrodes by Pediatric Cardiology. Subsequently, using a right subcostal laparotomy, and after intraoperative ultrasound, we conducted a subsegmental resection of the lesions in S 7 and S 8 with verification of the resection margins and fulguration of the bed with diathermy, resulting in intraoperative bleeding of less than 100cc. The patient was discharged on the 10th postoperative day, presenting mild decompensation of heart failure with good response to diuretic treatment.

The result of the pathological study of both nodules was moderately differentiated HCC, with no vascular invasion, and free margins. The non-tumorous parenchyma showed a markedly congestive cirrhotic pattern. After 12 months of follow-up, the patient continues to be asymptomatic, and no new suspicious lesions have been observed.

Most liver diseases are due to inflammatory damage caused in the periportal areas of the hepatic lobule. In patients with FP, the damage is caused by hepatic congestion that, as in other chronic liver diseases, leads to advanced fibrosis with the complications of portal hypertension, liver failure, and increased risk of HCC. These complications are time-dependent3,4.

Regenerative nodules, as in other congestive liver diseases, are very common in these patients. They are generally large, hypervascular, and located in the hepatic periphery. Some of these features are common to HCC. For this reason, the diagnosis of HCC in patients treated with FP requires biopsy for histological confirmation in most cases5–8.

There are few publications about the management of HCC in patients with FP, and most show a preference of non-surgical approaches (radioembolization, transcatheter arterial chemoembolization, or percutaneous ablation). Surgical resection is proposed as a feasible but limited alternative on many occasions, because of both the cardiac and hepatic functionality6,9. Definitive treatment could involve liver or heart-liver transplantation, which is contraindicated in pulmonary hypoplasia, as in this case.

Specialized patient follow-up is essential, along with the implementation of an HCC screening program after FP. Although the time to initiate screening has not been defined, most tumors appear at least 10 years after FP6. At our hospital, screening is indicated 10 years after FP, which includes a hepatic ultrasound every 6 months.

In cases where percutaneous procedures cannot be performed, surgical resection is a feasible, and potentially curative, alternative (Table 1).

Table 1.

Surgical resection in HCC after FP.

Main author  Year  Age  Sex  Complications of PHTCirrhosis  Time since FP (years)  Image  AFP  Stage (BCLC, Milan)  Treatment  Survival 
Payno  2022  32  Complications of PHT: NOCirrhosis: Yes  28  30 mm and 15 mm  7 ng/mL  BCLC A  Surgical resection  Alive after 12 months 
Yokota10  2020  18  Complications of PHT: NOCirrhosis: Yes  12  NR  3 ng/mL  BCLC AMilan: Yes  Laparoscopic resection  Alive after 12 months 
Nemoto11  2020  37  Complications of PHT: NOCirrhosis: Yes  31  80 mm  81.663 ng/mL  BCLC: AMilan: NO  Left hepatectomy  Alive after 16 months 
Angelico, Int J Surg Case Rep. 59:144−147.  2019  33  Complications of PHT: NOCirrhosis: Yes  27  45 mm  3.005 ng/mL  BCLC: AMilan: YES  Laparoscopic resection. Recurrence: TACE + Sorafenib  Alive after 7 months 
Lo,Transl Gastroenterol Hepatol. 3:51.  2018  24  Complications of PHT: NOCirrhosis: Yes  23  68 mm and 27 mm  50.000 ng/mL  BCLC: BMilan: NO  Left hepatectomy.Recurrence: TACE + Sorafenib  Exitus por cáncer a los 6 months 
Takuma,Intern Med. 55(22):3265−3272.  2016  29  Complications of PHT: AscitisCirrhosis: Yes  19  18 mm and 15 mm  117 ng/mL  BCLC: AMilan: YES  Surgical resection  Alive after 12 months 
Jitta, Int J Cardiol. 206:21−26.  2016  42  Complications of PHT: NOCirrhosis: Yes  32  NR  2.996 ng/mL  BCLC: AMilan: NO  Surgical resection  Alive after 12 months 
Dorsey, J Cardiothorac Vasc Anesth. 30(2):452−454.  2016  32  Complications of PHT: NOCirrhosis: Yes  23  40 mm  13 ng/mL  BCLC: AMilan: YES  Surgical resection  Alive after 12 months 
Maeda, Reg Anesth Pain Med. 40(6):718−719.  2015  12  Complications of PHT: NOCirrhosis: NR  10  12 mm  NR  BCLC: AMilan: YES  Surgical resection  NR 
Weyker, A A Case Rep. 2(8):99−101.  2014  23  Complications of PHT: NRCirrhosis: NR  22  148 mm  NR  BCLC: CMilan: NO  Surgical resection  NR 

M: male; F: female; PHT: portal hypertension; FP: Fontan procedure; AFP: alfafetoprotein; NR: not reported; TACE: transarterial chemoembolization.

Table modified by Rodriguez-Santiago E, Téllez L, Guerrero A, Albillos A. Hepatocellular carcinoma after Fontan surgery: A systematic review. Hepatol Res. 20216.

References
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[2]
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[3]
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Hepatocellular carcinoma after Fontan surgery: A systematic review.
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Int J Cardiol, 322 (2021), pp. 142-148
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[10]
Y. Yokota, T. Noda, S. Kobayashi, et al.
A case report of Fontan procedure-related hepatocellular carcinoma: Pure laparoscopic approach by low and stable pneumoperitoneum.
[11]
S. Nemoto, S.I. Ariizumi, Y. Kotera, A. Omori, S. Yamashita, T.-A. Kato, et al.
A patient with post-Fontan operation underwent left hepatectomy and caudate lobectomy for hepatocellular carcinoma: a case report.
Surg Case Rep, 19 (2020), pp. 104
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