Risk factors and serological markers of liver cirrhosis after Fontan procedure
Risk factors and serological markers of liver cirrhosis after Fontan procedure
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DOI：
10.1007/s00380-015-0743-4
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发表时间：
2016-09-01
影响因子：
1.5
通讯作者：
Nakanishi, Toshio
中科院分区：
文献类型：
--
作者：
Shimizu, Mikiko;Miyamoto, Kenji;Nakanishi, Toshio
肝硬化（LC）可能导致肝衰竭或癌症，已报告在Fontan手术后的患者中发生。本研究的目的是阐明LC的发生率和组织学特征，并评估LC伴Fontan循环的危险因素和血清学标志物。回顾性分析了57例Fontan手术后患者的肝脏CT增强扫描（CT）。患者分为两组：LC组（n = 31）和非LC组（n = 26）。比较两组Fontan手术时的年龄、Fontan手术后的持续时间、导管插入数据和Fontan循环衰竭史。比较血清学资料，包括γ-GTP和透明质酸。可用时，评估尸检标本的组织学。LC组Fontan手术后持续时间明显长于非LC组。LC组Fontan循环衰竭史较非LC组多见。在该系列中，手术类型（APC/Bjork/侧向隧道/TCPC）与LC之间没有相关性。LC组血清透明质酸、γ-GTP和Forns指数明显高于对照组。LC的重要危险因素是Fontan手术后的持续时间（> 20年）。在尸检标本中，LC的组织病理学变化主要见于中心静脉区。CT诊断LC多见于Fontan术后较长时间，尤其是20年后。透明质酸和γ-GTP可能是监测Fontan患者肝纤维化进展的有用标志物。
Liver cirrhosis (LC), which may result in hepatic failure or cancer, has been reported in patients after Fontan procedure. The purpose of this study was to clarify the frequency and histological characteristics of LC, and to evaluate the risk factors and serological markers of LC with Fontan circulation. Retrospective review of contrast-enhanced CT scans (CT) of the liver was carried out in 57 patients after Fontan procedure. Patients were divided into two groups: LC group (n = 31) and no LC group (n = 26). Age at Fontan procedure, duration after Fontan procedure, catheterization data, and history of failing Fontan circulation were compared between groups. Serological data including gamma-GTP and hyaluronic acid were compared. Histology of autopsy specimens was assessed when available. Duration after Fontan procedure was significantly longer in LC group than no LC group. History of failing Fontan circulation was more frequent in LC group than in no LC group. There was no correlation between type of procedure (APC/Bjork/lateral tunnel/TCPC) and LC in this series. Serum hyaluronic acid, gamma-GTP, and Forns index were significantly higher in LC group. Significant risk factors for LC were duration after Fontan procedure (> 20 years). In autopsy specimens, histopathological changes of LC were observed predominantly in the central venous area. LC diagnosed with CT is frequent in patients long after Fontan procedure, especially after 20 years. Hyaluronic acid and gamma-GTP could be useful markers to monitor the progression of liver fibrosis in Fontan patients.