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

Mohit Kehar1, Carolina Jimenez-Rivera1

  • 1From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Eastern Ontario, Department of Pediatrics, Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.

JPGN Reports
|May 11, 2023
PubMed

Insights

Practices for managing Fontan-Associated Liver Disease (FALD) in children vary significantly among Canadian pediatric hepatologists. There is a need for standardized protocols to ensure consistent care for these complex patients.

Area of Science:

  • Pediatric Hepatology
  • Cardiology
  • Congenital Heart Disease Management

Background:

  • Fontan-Associated Liver Disease (FALD) is a frequent complication after the Fontan procedure.
  • Current management and screening guidelines for FALD in children are lacking.
  • Variability in care impacts patient outcomes.

Purpose of the Study:

  • To assess current practices of Canadian pediatric hepatologists in managing FALD.
  • To identify variations in FALD care across academic centers in Canada.

Main Methods:

  • A nationwide electronic survey was distributed to pediatric hepatologists in university-affiliated hospitals.
  • The Canadian Pediatric Hepatology Research Group infrastructure was utilized.
  • Data were collected on screening, diagnostic modalities, and follow-up protocols.

Main Results:

  • Twelve hepatologists from 13 centers (92% response rate) participated.
  • Only 17% of centers have multidisciplinary post-Fontan care teams.
  • Significant differences were observed in screening, fibrosis assessment, varices screening, follow-up frequency, and transition planning.

Conclusions:

  • Substantial discrepancies exist in Canadian pediatric hepatologists' management of FALD.
  • A standardized protocol is essential for optimizing care for children and adolescents with FALD.
  • Further research is required to develop evidence-based guidelines.

Keywords:
Canadacardiaccongenital heart diseasehepatic diseasepediatric

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