Kogiso Tomomi, Tokuhara Daisuke, Ohfuji Satoko, Tanaka Atsushi, Kanto Tatsuya
Department of Internal Medicine and Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan.
Department of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Hepatol Res. 2025 Apr;55(4):611-621. doi: 10.1111/hepr.14141. Epub 2024 Nov 27.
Fontan-associated liver disease (FALD) is a complication after Fontan surgery, and a common cause of liver tumors and cirrhosis. However, no diagnostic criteria for FALD have been established, leading to an underestimation of its prevalence.
We conducted a national survey to elucidate the characteristics of FALD by collecting data from high-volume centers managing patients who had undergone the Fontan surgery in Japan. In total, 1168 patients were enrolled in the study. First, we examined typical liver findings on ultrasonography after the Fontan surgery. Next, we proposed diagnostic criteria for FALD and advanced FALD based on blood tests, imaging, liver tumors, and pathological examinations. We investigated the sensitivity of histologically diagnosed FALD and advanced FALD based on criteria for blood or imaging tests.
Hepatomegaly, hepatic venous dilatation, caudate lobe enlargement, splenomegaly, liver atrophy, ascites, hepatocellular carcinoma, and hepatic tumors other than hepatocellular carcinoma were observed in 37.7%, 29.9%, 18.4%, 33.2%, 3.2%, 6.0%, 0.85%, and 10.0% of patients, respectively. Typical ultrasound findings of FALD included hepatomegaly, hepatic vein dilatation, and splenomegaly, reflecting liver congestion. With the progression of fibrosis, caudate lobe enlargement and splenomegaly became more prominent. Based on these findings, we proposed diagnostic criteria for FALD. Using these criteria, FALD was diagnosed in 1014 (86.8%) of the patients, and all patients with a pathological diagnosis of FALD were successfully identified. Eight patients were found to have pathological cirrhosis, and all were diagnosed with advanced FALD using our criteria based on blood tests or imaging.
Our diagnostic criteria facilitate detection of FALD or advanced FALD after the Fontan surgery. The accuracy of these criteria should be further evaluated.
Fontan相关肝病（FALD）是Fontan手术后的一种并发症，是肝肿瘤和肝硬化的常见病因。然而，尚未建立FALD的诊断标准，导致其患病率被低估。
我们进行了一项全国性调查，通过收集日本大量管理Fontan手术患者的中心的数据来阐明FALD的特征。共有1168例患者纳入本研究。首先，我们检查了Fontan手术后超声检查的典型肝脏表现。接下来，我们基于血液检查、影像学、肝肿瘤和病理检查提出了FALD和晚期FALD的诊断标准。我们根据血液或影像学检查标准调查了组织学诊断的FALD和晚期FALD的敏感性。
分别在37.7%、29.9%、18.4%、33.2%、3.2%、6.0%、0.85%和10.0%的患者中观察到肝肿大、肝静脉扩张、尾状叶增大、脾肿大、肝萎缩、腹水、肝细胞癌和非肝细胞癌的肝肿瘤。FALD的典型超声表现包括肝肿大、肝静脉扩张和脾肿大，反映肝脏充血。随着纤维化的进展，尾状叶增大和脾肿大变得更加明显。基于这些发现，我们提出了FALD的诊断标准。使用这些标准，1014例（86.8%）患者被诊断为FALD，所有经病理诊断为FALD的患者均被成功识别。发现8例患者有病理肝硬化，所有患者根据我们基于血液检查或影像学的标准被诊断为晚期FALD。
我们的诊断标准有助于Fontan手术后FALD或晚期FALD的检测。这些标准的准确性应进一步评估。
