Takashi Motomura, Shinji Itoh, Takeo Toshima, Shohei Yoshiya, Kyohei Yugawa, Norifumi Iseda, Tomoharu Yoshizumi
BACKGROUND: Complications associated with the Fontan circulation have become evident in recent years. Fontan-associated liver disease (FALD) has attracted particular attention. However, many aspects of this condition including FALD-associated hepatocellular carcinoma (FALD-HCC) remain unclear. PATIENTS AND METHODS: Since 2018, 12 liver resections for FALD-HCC were performed in 9 patients at our institution. These 12 procedures were reviewed, and 9 cases of primary HCC were compared with 216 cases of primary HCC resection performed during the same period in non-FALD patients in terms of short- and long-term outcomes. RESULTS: Among 12, nine were initial and three were recurrences. Approaches included open (n = 6), laparoscopic (n = 4), and robotic surgery (n = 2). Compared with non-FALD patients, FALD group was younger (37 vs. 69 years, p < 0.0001), had lower BMI (19.0 vs. 24.1, p = 0.0002) and showed advanced fibrosis/cirrhosis (F3-4) more frequently (p = 0.03). Intraoperative blood loss was greater in the FALD group (1695 vs. 520 mL, p = 0.0003). Factors associated with blood loss > 1000 mL included CVP > 12 mmHg, tumor size > 2 cm, tumor depth > 3 cm from the liver surface. Among nine patients under regular post-Fontan imaging surveillance, tumors tended to be smaller and blood loss lower. With a mean follow-up period of 32.1 months in the FALD group, recurrence-free survival did not differ between the groups. CONCLUSION: Long-term outcomes seem comparable between FALD and non-FALD HCC. In particular, FALD-HCC ≤ 2 cm and close to the liver surface with CVP ≤ 12 mmHg could be potential candidates for surgical resection, including minimally invasive approaches. Post-Fontan surveillance may be crucial.