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Early Experience with the First Canadian Paediatric Fontan Long-Term Follow-up Clinic.
Sang-Yun Lee1,2, Lauren Scavuzzo2, Stephanie Darlington2
1Department of Pediatrics, College of Medicine, Seoul National University, Seoul National University Children's Hospital, Seoul, Republic of Korea.
Pediatric Cardiology
|March 4, 2026
Summary
Patients undergoing the Fontan procedure face significant long-term health issues beyond cardiac function, including liver, blood, bone, and developmental problems. Comprehensive, ongoing multidisciplinary care is crucial for managing these Fontan-associated morbidities and improving quality of life.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Long-term Outcomes
Background:
- The Fontan procedure improves survival for single-ventricle physiology but leads to multisystem complications.
- Multisystem complications are a major cause of late morbidity and mortality in Fontan patients.
- A dedicated pediatric Fontan Long-Term Follow-up Clinic was established to address these issues.
Purpose of the Study:
- To evaluate the prevalence and spectrum of cardiac and extracardiac morbidities in a dedicated Fontan clinic cohort.
- To assess the health status of patients post-Fontan completion through standardized evaluations.
- To identify common complications and inform long-term management strategies.
Main Methods:
- Single-center retrospective study of patients referred to the Fontan Long-Term Follow-up Clinic (January 2020 - December 2024).
- Standardized cardiac assessments (echocardiography, Holter, exercise testing, MRI) and extracardiac investigations (hepatic, renal, hematologic, endocrine, growth, neurodevelopmental).
- Fontan-associated liver disease (FALD) graded using MR elastography and imaging.
Main Results:
- 161 patients assessed (mean age 13.2 years, 9.5 years post-Fontan).
- Cardiac function generally preserved (mean predicted peak VO₂ 63.6%), but moderate/severe atrioventricular valve regurgitation (21.5%) and ventricular dysfunction (5.6%) noted.
- High prevalence of extracardiac morbidities: FALD (8.0%), lymphopenia (64.6%), vitamin D deficiency (41.9%), reduced bone density (28.0%), and neurodevelopmental challenges (39.8%).
Conclusions:
- Despite preserved cardiac performance, Fontan patients experience a high burden of hepatic, hematologic, endocrine, skeletal, and neurocognitive morbidities.
- Fontan-associated liver disease is present even with preserved cardiac function.
- Multidisciplinary, longitudinal follow-up is essential for optimizing outcomes and quality of life in Fontan survivors.
