Abstract

BackgroundThe Fontan procedure has been widely accepted for children with single ventricle physiology and guarantees survival rates of approximately 80% at age 20 years. However, there have been cases of Fontan-associated liver disease (FALD) caused due to congestion, along with recent reports of the development of hepatocellular carcinoma (HCC) in younger patients with FALD. The literature consists of only five previous case reports of patients who underwent hepatectomy for HCC due to poorer cardiac function and liver cirrhosis caused due to congestion.Case presentationThe patient was a 37-year-old woman who presented with epigastralgia. Computed tomography (CT) revealed a liver tumor, 8 cm in diameter, in the caudate lobe. Liver damage was A, with an indocyanine green retention rate of 6% at 15 min. The levels of alpha-fetoprotein (AFP) and protein induced by vitamin K antagonists-II (PIVKA-II) were elevated to 81,663 ng/ml (normal < 10 ng/ml) and 238 mAU/ml (normal < 40 mAU/ml), respectively. Left ventricular ejection fraction was 56%, and central venous pressure (CVP) was 12 mmHg. Left hepatectomy and caudate lobe resection were successfully performed in the reverse Trendelenburg position which reduced the CVP. The total operation duration was 450 min, with a total blood loss of 3200 ml. The patient’s postoperative course was uneventful, and she is still alive 16 months after surgery.ConclusionsFirst left hepatectomy with caudate lobectomy during reverse Trendelenburg position which reduced the CVP was performed in a patient with HCC and FALD.

Highlights

  • The Fontan procedure has been widely accepted for children with single ventricle physiology and guarantees survival rates of approximately 80% at age 20 years

  • First left hepatectomy with caudate lobectomy during reverse Trendelenburg position which reduced the central venous pressure (CVP) was performed in a patient with hepatocellular carcinoma (HCC) and Fontanassociated liver disease (FALD)

  • It has been reported that Fontan-associated liver disease (FALD) develops due to congestion with the growth of these children who survive for a long time

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Summary

Conclusions

First left hepatectomy with caudate lobectomy during reverse Trendelenburg position which reduced the CVP was performed in a patient with HCC and FALD. Abbreviations CVP: Central venous pressure; FC: Fontan circulation; AV: Atrioventricular; rTP: Reverse Trendelenburg position; cIVC: Inferior vena cava clamp; AFP: Alpha-fetoprotein; TACE: Transarterial chemoembolization; TAE: Transarterial embolization

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