Care Pattern for Fontan-Associated Liver Disease by Academic Pediatric Hepatologists in Canada

Fontan-Associated Liver Disease (FALD) is a common extracardiac complication seen in patients following the Fontan procedure. There are no consensus guidelines on screening and management of children with FALD. OBJECTIVE: The current study aims to determine academic pediatric hepatologists’ practices and identify variability in management provided to children with FALD in Canada. METHODS: Using the infrastructure of the Canadian Pediatric Hepatology Research Group, a nationwide survey was distributed electronically to all pediatric hepatologists practicing in university-affiliated hospitals. RESULTS: Twelve pediatric hepatologists from 12 of 13 academic centers (92%) responded to the survey. The institutions of only 2 (17%) physicians offer post-Fontan care with a multidisciplinary team, both from different provinces. The screening for other comorbidities, use of noninvasive modality, and timing of liver biopsy for estimation of liver fibrosis and screening for esophageal varices differ from program to program. The frequency of outpatient clinic follow-up varies significantly. Education and counseling concerning liver health are generally used as treatment; only 58% of academic centers have a formal adult care transition plan. CONCLUSIONS: Significant discrepancies exist in the care provided to children with FALD by hepatologists practicing in academic centers across Canada. Future study is needed to develop a standardized protocol for managing and following children and youth with FALD.