a Department of Pediatrics , Toho University, Sakura Medical Center , Chiba , Japan.
b Department of Pediatric Hepatology and Gastroenterology , Eastern Yokohama Hospital , Kanagawa , Japan.
Expert Rev Gastroenterol Hepatol. 2019 Jul;13(7):651-666. doi: 10.1080/17474124.2019.1621746. Epub 2019 May 26.
: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation. : The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed. : Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.
肝纤维化和肝细胞癌（HCC）可发生于先天性心脏病患儿。虽然 Fontan 手术后易发生肝纤维化和 HCC，但未行 Fontan 手术的先天性心脏病患者也可发生。本文描述了包括 Fontan 相关肝病在内的心脏肝病史。回顾患者特征、肝脏组织学、影像学检查和血液检查结果，以阐明心脏肝病的发病机制。此外，还提出了先天性心脏病患儿心脏肝病随访管理的流程图。充血和低心输出量是心脏肝病的主要原因。晚期肝纤维化被认为与 HCC 相关。HCC 可发生于青少年和年轻成人。无论是否行 Fontan 手术，功能性单心室和慢性心力衰竭的患儿都应定期检查心脏肝病。目前尚无单一可靠的实验室参数可准确检测心脏肝病；纤维化指标和弹性成像对该病的检测结果不一致。需要使用肝脏标本证实的患者进行进一步研究，并规范评估方案，以阐明心脏肝病的发病机制。
