Fontan相关性肝病的现状与移植考虑

Fontan-associated liver disease current status and transplantation consideration

Fontan相关性肝病(FALD)是Fontan术后的主要并发症之一,主要表现为肝纤维化甚至是肝硬化,发病率很高,临床上缺乏典型症状,严重影响患者预后。确切的病因未明,被认为与长期的中心静脉压升高和肝动脉血流受损等因素相关。实验室检查及影像学资料与肝纤维化严重程度之间缺乏相关性,导致临床诊断和监测存在困难。肝活检是肝纤维化诊断及分期的金标准。Fontan术后时间是FALD最重要的危险因素,建议在Fontan手术10年后进行肝活检,同时警惕肝细胞癌的存在。对于存在Fontan循环衰竭合并晚期肝纤维化的患者,联合心脏-肝移植是一种优先的选择,取得了较好的结果。

Fontan-associated liver disease(FALD)is one of the main complications after the Fontan procedure,manifesting mostly as liver fibrosis and even cirrhosis,with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis.The specific cause is unknown,although it is considered to be associated with long-term elevated central venous pressure,impaired hepatic artery blood flow,and other relevant factors.The absence of association between laboratory tests,imaging data,and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult.A liver biopsy is the gold standard for diagnosing and staging liver fibrosis.The most important risk factor for FALD is time following the Fontan procedure;therefore,it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma.Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.