Predictors of Adverse Liver-Related Outcomes in Adults After the Fontan Operation

Understanding Fontan-Associated Liver Disease—The Role of Tools for Noninvasive Assessment

“…While FALD remains indolent in the majority of patients, some Fontan patients develop hepatic decompensation or hepatocellular carcinoma or require heart–liver transplant, particularly in adulthood. 2 Liver injury in Fontan patients seems to be multifactorial and related to chronic venous congestion in combination with low cardiac output producing hypoxic stress exacerbated by perioperative insults and acute or chronic injury that may trigger inflammation and subsequent liver fibrosis or cirrhosis. 3 Pathologic data suggest that liver disease in this population is the result of both pre- and post-Fontan liver damage, making it difficult to target specific insults.…”