Higher LSM is associated with unfavorable Fontan hemodynamics and advanced centrilobular hepatic fibrosis. TE may be a useful tool for identifying Fontan patients who warrant invasive testing.

In this largest reported pediatric PSC cohort, liver transplantation rate at 10 years was lower than that reported in adults. ASC and PSC had similar biochemical abnormalities and degree of fibrosis at diagnosis. PSC that developed after IBD diagnosis had a milder course, possibly reflecting earlier disease detection or milder phenotype.