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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.
Insights
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.
Abstract:
The Fontan procedure has significantly improved survival in patients with single-ventricle physiology; however, multisystem complications have become a major source of late morbidity and mortality. In 2020, the Hospital for Sick Children (SickKids) established a dedicated paediatric Fontan Long-Term Follow-up Clinic. This study evaluated the prevalence and spectrum of cardiac and extracardiac morbidities in the clinic cohort since inception. This single-centre retrospective study included all patients referred between January 2020 and December 2024. Standardized assessments comprised cardiac evaluations (echocardiography, Holter monitoring, exercise testing, cardiac MRI) and extracardiac investigations of hepatic, renal, hematologic, endocrine, growth, and neurodevelopmental status. Fontan-associated liver disease (FALD) was graded using MR elastography and imaging findings. A total of 161 patients (mean age 13.2 ± 3.7 years; 58.4% male) were assessed at a mean of 9.5 ± 4.3 years post-Fontan completion. Tricuspid atresia was the most common diagnosis (26.1%). Cardiac function was generally preserved, with mean predicted peak VO₂ of 63.6 ± 17.6%. Moderate or severe atrioventricular valve regurgitation occurred in 21.5%, and ventricular dysfunction in 5.6%. Moderate or greater FALD was observed in 8.0%, with mean magnetic resonance elastography 4.58 kPa (Interquartile range, 4.03-5.40). Lymphopenia (64.6%), thrombocytopenia (16.8%), vitamin D deficiency (41.9%), and reduced bone density (28.0%) were frequent. Neurodevelopmental challenges were common (39.8% learning difficulties). Despite preserved cardiac performance, Fontan patients exhibited a high burden of hepatic, hematologic, endocrine, skeletal, and neurocognitive morbidities. Multidisciplinary, longitudinal follow-up is essential to optimize outcomes and quality of life.
