パネルディスカッション1（I-PD01）
より良いフォンタンの生涯に向けて

[I-PD01-01] Fontan術後肝障害の予後と危険因子：全国サーベイランス結果

^○新居 正基¹, 中西 敏雄², 犬塚 亮³, 「単心室循環症候群の治療管理の質を高めるための研究」研究班 (1.静岡県立子ども病院 循環器科, 2.東京女子医科大学医学部循環器小児科, 3.東京大学医学部付属病院小児科)

Keywords:Fontan手術, 肝障害, 肝硬変

【Introduction】Fontan associated liver disease (FALD) is increasingly recognized as a common complication in patients late after Fontan operation (FO). Although many reports revealed the correlation between the extent of liver fibrosis (LF) and hepatic venous pressure or duration after FO, some reports suggest the existence of LF even before FO. There is still paucity of data regarding the incidence and risk factors of FALD. 【Objectives】As there are many variations of LF and focal nodular hyperplasia related to FALD, and they are difficult to be diagnosed correctly at an early stage, analysis was focused on liver cirrhosis (LC) and hepatocellular carcinoma (HCC), which are clinically more important. Our objectives are twofold: 1) to elucidate the incidence of LC and HCC in Japan; 2) to reveal the risk factors causing LC or HCC.【Methods】The questionnaires were sent to 12 major cardiac centers in Japan between 2016 and 2017. All the patients who had FO before 2010 and discharged alive were included in the survey. LC and HCC were diagnosed based on the clinical, laboratory, imaging, and biopsy findings. 【Results】1286 patients were included in this study (male: n = 669 (52%); age: median: 3.6 (range: 0.3-52.8)). There were 50 (3.9%) patients with LC and 8 (0.6%) patients with HCC. Incidence of probability of LC and HCC increases over the years after FO: at 20 years LC=9% / HCC=0.7%; at 40 years: LC=64% / HCC=7%. Mortality of all causes at 5 years after the diagnosis of LC or HCC were 24% and 50%, respectively. The risk factors to develop LC were: corrected transposition of great arteries, pulmonary atresia with intact septum, atrioventricular septal defect, heterotaxy syndrome, pulmonary arterial index (Nakata index) before FO, and cardiac index after FO on multivariate analysis.【Conclusion】Incidence of LC and HCC increases over the years after FO. In addition to the post-FO cardiac output, the cardiac anatomy or pre-FO hemodynamic indices are also the risk factors to develop FALD.