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

**Background:** Fontan-Associated Liver Disease (FALD) is one of the most common extracardiac complications following the Fontan procedure, which is the preferred surgical palliation for single ventricle congenital heart disease. Virtually 100% of post-Fontan patients have some degree of hepatic fibrosis, with bridging fibrosis reported in nearly 40% by adolescence. Despite growing awareness, FALD screening and management guidelines are lacking in the pediatric population, and no data were previously available on current practice patterns in Canada.

**Methods:** Using the infrastructure of the Canadian Pediatric Hepatology Research Group, a nationwide anonymous online Redcap survey was distributed to all academic pediatric hepatologists across 13 academic pediatric centers in Canada from September 2021 to December 2021. A 48-item questionnaire covering respondent information, referral status and care pattern, monitoring, assessment, treatment patterns, and transition of care was developed based on literature review and consensus. The survey was pilot tested at Children's Hospital of Eastern Ontario. Descriptive statistics were employed using Stata v11.

**Key Results:** Twelve of 21 pediatric hepatologists (57%) from 12 of 13 (92%) academic centers responded. Only 2 physicians (17%) have a multidisciplinary team for post-Fontan care, both from different provinces. The majority (11/12, 92%) see one new FALD referral per year, with a median of 3 (range 2–10) patients followed. At clinic visits, the most common tests were complete blood count, prothrombin time/INR, liver panel, and HCC screening. HCC screening is performed by ultrasound (12/12) and alpha-fetoprotein (11/12). Abdominal ultrasound is the most common imaging modality (12/12, 100%), followed by MRI (4/12) and CT (2/12). Liver biopsy is performed at 8 of 12 centers, with timing varying from heart transplant evaluation (4 centers) to 10 years post-Fontan (2 centers). Annual transient elastography (Fibroscan) is used by 8/12 (67%) of respondents. No respondents use serum biomarkers for fibrosis monitoring. Variceal screening is considered by 9 respondents depending on clinical status, while 3 centers do not perform variceal screening. Follow-up frequency varies: 5 (42%) follow annually, 3 every 6 months, 1 every 3 months, 1 every 2–3 years, and 1 does not follow beyond the first visit. Ten (83%) counsel on risky behavior and diet/obesity/NAFLD. Only 7 (58%) have a formal transition program to adult care.

**Clinical Implications:** This first-ever assessment of Canadian academic pediatric hepatologists' FALD practices reveals substantial variability across all domains of care, including screening, monitoring, biopsy timing, variceal screening, follow-up frequency, and transition planning. The lack of multidisciplinary clinics (only 17% of centers) and formal transition programs (58%) represents critical gaps. The authors propose a standardized monitoring and counseling framework based on age stratification (child <10 years vs. adolescent 10–18 years), including suggested visit frequency, lab monitoring, imaging intervals, and biopsy indications. Given that time since Fontan surgery is the main risk factor (4-fold increased odds of hepatic complications at 11–15 years and 9-fold at 16–20 years post-surgery), standardized surveillance protocols are urgently needed to improve outcomes for this growing population of pediatric FALD patients.