Laparoscopic liver resection for hepatocellular carcinoma in Fontan-associated chronic liver disease. The first case report

INTRODUCTION: A well-recognized long-term complication after Fontan procedure (FP), a complex cardiac surgery performed in patients with univentricular hearts, is the development of chronic liver disease and hepatocellular carcinoma (HCC). Due to the risk of cardiac and liver decompensation, liver resection of HCC is challenging and the laparoscopic approach has never been reported. PRESENTATION OF THE CASE: We present the first case of laparoscopic liver resection (LLR) of HCC in a 33-years-old girl with cardiac-related cirrhosis after FP. Intraoperatively, the pneumoperitoneum was established at 8–10 mmHg and adequate fluid infusion was given to maintain the cardiac preload. After an ultrasound-guided thermoablation along the free-tumor margin of the hepatic lesion, a full laparoscopic non-anatomical resection of the tumor in segment V was performed, without Pringle manouver and blood transfusion requirement. The cardiac function remained stable during the surgery and thereafter, and the post-operative course was uneventful. DISCUSSION: HCC in chronic liver disease after FP is associated with high-risk mortality. Due to the complex hemodynamic changes after FP, open surgical resections often aren’t feasible and loco-regional percutaneous treatment or combined liver-heart transplantation are the only therapeutic options. This case suggests that LLR in FP patients has low-risk of liver and cardiac decompensation, minimizing the pneumoperitoneum insufflation to ensure low intra-abdominal/intra-thoracic pressures and providing accurate anaesthetic management to maintain proper cardiac preload and output. CONCLUSION: LLR for HCC after FP is safe and feasible, and might be considered an alternative treatment of HCC for which the best treatment has not been defined yet.