Identifying predictors of hepatic disease in patients after the Fontan operation: A postmortem analysis

Objective We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease. Methods From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis. Results The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal ( P