Ohfuji Satoko, Tanaka Atsushi, Kogiso Tomomi, Kanto Tatsuya
Department of Public Health, Osaka Metropolitan University Graduate School of Medicine, Osaka, Japan.
Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Hepatol Res. 2024 Oct;54(10):931-941. doi: 10.1111/hepr.14040. Epub 2024 Mar 25.
Although the Fontan procedure has improved the survival of patients with single-ventricle heart disease, the long-term consequences of the procedure have been a concern. This study aimed to explore the patients' postoperative clinical characteristics, including a diagnosis of Fontan-associated liver disease (FALD).
A nationwide Japanese epidemiological survey of post-Fontan patients was undertaken in 2021. The survey targets were selected from all departments of pediatrics, pediatric surgery, cardiology, cardiovascular surgery, and gastroenterology using stratified random sampling by the number of beds. Each department was asked to complete a mail-back questionnaire on the numbers of patients and their clinical characteristics. The diagnosis of FALD was made by each attending physician.
The estimated number of post-Fontan patients was 7810 (95% confidence interval, 5430-10 200) in 2020, with a period prevalence of 61.9 per million. During the follow-up of 13.8 years after the Fontan procedure, 40% of patients were diagnosed with FALD. An elevated γ-glutamyl transpeptidase level was the most common finding leading to the FALD diagnosis (41%), and 45% of the patients also showed liver fibrosis. Compared with non-FALD patients, FALD patients were older, had longer duration since the Fontan procedure, and had more severe cardiac or liver conditions. However, more than half of the non-FALD patients had elevated liver enzyme levels, suggesting underestimation of the number of FALD patients.
In 2020, approximately 40% of post-Fontan patients underwent follow-up with a diagnosis of FALD, although the lack of established diagnostic criteria for FALD could affect the reported prevalence of FALD.
尽管Fontan手术提高了单心室心脏病患者的生存率，但该手术的长期后果一直令人担忧。本研究旨在探讨患者术后的临床特征，包括Fontan相关肝病（FALD）的诊断。
2021年在日本开展了一项针对Fontan术后患者的全国性流行病学调查。调查对象通过按病床数量进行分层随机抽样，从儿科、小儿外科、心脏病学、心血管外科和胃肠病学的所有科室中选取。要求每个科室填写一份关于患者数量及其临床特征的回邮问卷。FALD的诊断由每位主治医生做出。
2020年Fontan术后患者的估计数量为7810例（95%置信区间，5430 - 10200），期间患病率为每百万61.9例。在Fontan手术13.8年的随访期间，40%的患者被诊断为FALD。γ-谷氨酰转肽酶水平升高是导致FALD诊断的最常见发现（41%），45%的患者还表现出肝纤维化。与非FALD患者相比，FALD患者年龄更大，Fontan手术后的病程更长，心脏或肝脏状况更严重。然而，超过一半的非FALD患者肝酶水平升高，这表明FALD患者的数量可能被低估。
2020年，约40%的Fontan术后患者在随访中被诊断为FALD，尽管缺乏FALD的既定诊断标准可能会影响所报告的FALD患病率。
