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Long-term developmental outcome of children with complex congenital heart disease
1Division of Cardiology, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, USA. mahle@email.chop.edu
Insights
As survival rates for congenital heart disease (CHD) surgery improve, research now focuses on long-term neurocognitive outcomes in survivors. Most children with repaired CHD perform within normal ranges, even those with complex conditions like hypoplastic left heart syndrome (HLHS).
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Surgery
Background:
- Decreasing mortality in neonatal and infant surgery for congenital heart disease (CHD) shifts focus to long-term outcomes.
- Survivors of CHD surgery face an increased risk of neurocognitive and neurologic deficits.
- Understanding long-term sequelae is crucial for improving quality of life in CHD survivors.
Purpose of the Study:
- To evaluate the long-term cognitive and neurologic function in survivors of neonatal and infant surgery for CHD.
- To identify risk factors and compare neurocognitive outcomes across different CHD subtypes and patient populations.
- To inform strategies for reducing cerebral injury during and after cardiac surgery.
Main Methods:
- Review of existing literature and clinical data on neurocognitive outcomes in CHD survivors.
- Analysis of standardized neurocognitive assessments in children with repaired or palliated CHD.
- Comparison of neurocognitive performance between high-risk CHD patients (e.g., HLHS) and other pediatric surgical populations.
Main Results:
- Most survivors of CHD surgery perform within the normal range on most standardized neurocognitive measures.
- Children with repaired or palliated CHD demonstrate an elevated risk for neurocognitive deficits.
- Even high-risk patients, such as those with hypoplastic left heart syndrome (HLHS), show comparable neurocognitive function to survivors of other complex conditions or low-birth weight infants.
Conclusions:
- While neurocognitive deficits are a risk in CHD survivors, most achieve normal functional ranges.
- Long-term neurocognitive outcomes in CHD survivors are generally comparable to other high-risk pediatric populations.
- Ongoing research and clinical efforts aim to minimize cerebral injury to further improve neurodevelopmental outcomes in these children.
Abstract:
As the mortality of neonatal and infant surgery for CHD continues to decrease, attention is now focused on long-term sequelae, especially later cognitive and neurologic function, in survivors. Although children with repaired or palliated CHD have an increased risk for neurocognitive deficits, most survivors are performing within the normal range for most standardized measures. Even those children at highest risk, such as patients with HLHS, are comparable with survivors of other congenital lesions, such as diaphragmatic hernia, or low-birth weight children. Continued efforts are underway to reduce cerebral injury before, during, and after congenital heart surgery.