Fontan-associated liver disease current status and transplantation consideration.
10.3760/cma.j.cn501113-20210612-00277
- VernacularTitle:Fontan相关性肝病的现状与移植考虑
- Author:
Xiang LIU
1
;
Jia Zi Chao TU
1
;
Yun TENG
1
;
Ji Mei CHEN
1
Author Information
1. Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou 510080, China School of Medicine, South China University of Technology, Guangzhou 510006, China.
- Publication Type:Journal Article
- Keywords:
Fontan procedure;
Heart transplantation;
Liver disease;
Liver transplantation
- MeSH:
Humans;
Liver Diseases/pathology*;
Liver Cirrhosis/pathology*;
Liver/pathology*;
Carcinoma, Hepatocellular/pathology*;
Liver Transplantation/adverse effects*;
Fontan Procedure/adverse effects*;
Postoperative Complications/pathology*;
Liver Neoplasms/pathology*
- From:
Chinese Journal of Hepatology
2023;31(1):109-112
- CountryChina
- Language:Chinese
-
Abstract:
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.
