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Neurodevelopmental outcomes in preschool survivors of the Fontan procedure
J William Gaynor1, Richard F Ittenbach2, Marsha Gerdes3
1Division of Cardiac Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pa.
Insights
Preschool survivors of the Fontan procedure show similar neurodevelopmental outcomes to children with congenital heart disease undergoing biventricular repair. Patient factors, not surgical details, predict outcomes in single ventricle heart disease.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease
Background:
- The Fontan procedure is a palliative surgery for single ventricle congenital heart disease.
- Neurodevelopmental outcomes in survivors are a critical concern.
- Comparing outcomes after Fontan procedure versus biventricular repair is essential.
Purpose of the Study:
- To compare neurodevelopmental outcomes of preschool survivors of the Fontan procedure with those of children undergoing biventricular repair.
- To identify predictors of neurodevelopmental outcome in single ventricle heart disease patients, including hypoplastic left heart syndrome.
Main Methods:
- Neurodevelopmental outcomes assessed at 4 years: cognition, visual-motor integration, behavior, social skills, academic achievement.
- Comparison between single ventricle (post-Fontan) and biventricular repair groups.
- Multivariable analysis of patient, operative, and postoperative factors predicting outcomes in single ventricle patients.
Main Results:
- 365 children evaluated: 112 post-Fontan (91 hypoplastic left heart syndrome), 253 post-biventricular repair.
- Single ventricle patients showed worse processing speed, inattention, and impulsivity compared to biventricular repair.
- No significant differences in most domains; hypoplastic left heart syndrome outcomes not worse than other single ventricles. Patient factors were stronger predictors.
Conclusions:
- Preschool survivors of the Fontan procedure have largely similar neurodevelopmental outcomes to peers with biventricular repair.
- Hypoplastic left heart syndrome does not confer worse outcomes than other single ventricle conditions post-Fontan.
- Patient-related factors are more influential than surgical variables in predicting neurodevelopmental outcomes.
Objectives:
The study objectives were to compare the neurodevelopmental outcomes of preschool survivors of the Fontan procedure with those of children with congenital heart disease undergoing biventricular repair and to investigate predictors of neurodevelopmental outcome for those with single ventricle congenital heart disease, including hypoplastic left heart syndrome.
Methods:
Neurodevelopmental outcomes were assessed at 4 years of age, including cognition, visual-motor integration, behavior, social skills, and academic achievement. Unadjusted outcomes were compared between patients with biventricular circulation and patients with single ventricles. Predictors of neurodevelopmental outcome were assessed in the patients with single ventricles. Multiple covariate models were evaluated using patient-related, operative, and postoperative covariates.
Results:
Neurodevelopmental evaluation was performed in 365 children, 112 after the Fontan procedure (hypoplastic left heart syndrome, n = 91; other single ventricle, n = 21) and 253 after biventricular repair. Compared with patients with biventricular circulation, patients with single ventricles performed worse in terms of processing speed, inattention, and impulsivity. Otherwise, there were no significant differences between the groups for any domain. There was a trend toward lower performance for patients with single ventricles on visual motor integration. Outcomes for patients with hypoplastic left heart syndrome were not worse than for other forms of functional single ventricle. Patient factors were more important predictors of neurodevelopmental outcomes than were operative management variables.
Conclusions:
In this cohort, unadjusted neurodevelopmental outcomes for preschool survivors of the Fontan procedure are similar to those for children with congenital heart disease undergoing biventricular repair for most domains. Among the patients undergoing the Fontan procedure, hypoplastic left heart syndrome was not associated with worse outcomes compared with other forms of single ventricle.