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Updated: Jun 8, 2026

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Published on: May 2, 2025
Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including
Gerhard-Paul Diller1, Alessandro Giardini, Konstantinos Dimopoulos
1Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College of Science and Medicine, Sydney Street, SW3 6NP London, UK. g.diller@imperial.ac.uk
Exercise intolerance in Fontan patients is linked to hospitalizations, not death or transplant. Key risk factors for poor outcomes include arrhythmia and specific Fontan types.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Exercise intolerance is linked to worse outcomes in congenital heart disease.
- The Fontan procedure is a palliative surgery for complex single-ventricle defects.
- Identifying risk factors for Fontan patient outcomes is crucial.
Purpose of the Study:
- To determine if exercise intolerance predicts poor outcomes in Fontan patients.
- To identify risk factors for mortality, cardiac transplantation, and hospitalization in Fontan survivors.
Main Methods:
- Retrospective analysis of 321 Fontan patients undergoing cardiopulmonary exercise testing (CPET).
- Data collected from four European centers between 1997 and 2008.
- Follow-up for a median of 21 months to assess mortality, transplantation, and hospitalizations.
Main Results:
- CPET parameters, except heart rate reserve, correlated with hospitalization risk but not death or transplantation.
- Clinically relevant arrhythmia increased risk of death/transplantation 6-fold.
- Atriopulmonary/atrio-ventricular Fontan connections carried a 3.7-fold higher risk compared to total cavopulmonary connection.
- Combined risk factors (arrhythmia, specific Fontan type, heart failure) predicted a 25% 3-year mortality.
Conclusions:
- Most CPET parameters predict hospitalization, not mortality or transplantation, in contemporary Fontan patients.
- Decreased heart rate reserve, arrhythmia, atriopulmonary/atrio-ventricular Fontan, and heart failure indicate poor prognosis.
- These factors may identify Fontan patients needing closer medical or surgical management.
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