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Neurodevelopmental burden at age 5 years in patients with univentricular heart
Anne Sarajuuri1, Eero Jokinen, Leena Mildh
1Divisions of Child Neurology, Helsinki University Central Hospital and University of Helsinki, Helsinki, Finland. anne.sarajuuri@hus.fi
Insights
Children with hypoplastic left heart syndrome (HLHS) and univentricular heart defects (UVH) show neurodevelopmental deficits and brain abnormalities by age 5. Aortic size and surgical factors impact outcomes.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Pediatric Neurology
Background:
- Hypoplastic left heart syndrome (HLHS) and univentricular heart defects (UVH) are congenital heart conditions associated with increased neurodevelopmental risks.
- Despite improved survival rates, long-term neurodevelopmental outcomes in these patients require further investigation.
Purpose of the Study:
- To assess neurodevelopmental outcomes and brain MRI findings in children with HLHS and UVH at preschool age.
- To identify risk factors associated with neurodevelopmental deficits in these patient populations.
Main Methods:
- Prospective follow-up of 23 HLHS patients, 13 UVH patients, and 40 controls until age 5.
- Comprehensive assessments included neurologic, neuropsychological, and motor examinations, alongside brain MRI.
Main Results:
- Patients with HLHS and UVH exhibited significantly lower median full-scale IQ scores compared to controls.
- Major neurodevelopmental impairment and minor neurologic dysfunction were prevalent in both HLHS and UVH groups.
- Brain MRI revealed abnormalities, primarily ischemic changes, in a majority of HLHS and UVH patients, correlating with neurodevelopmental findings.
Conclusions:
- Neurodevelopmental and brain MRI abnormalities are common in children with HLHS and UVH by preschool age.
- A narrowed ascending aorta and factors related to surgical procedures contribute to adverse neurodevelopmental outcomes.
Background:
Despite increasing survival, patients with hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects (UVHs) remain at increased risk of long-term neurodevelopmental deficits.
Methods:
A nationwide sample of 23 patients with HLHS, 13 with UVH, and 40 controls were followed prospectively until the age of 5 years, when neurologic, neuropsychological, and motor examinations and brain MRI were performed.
Results:
The median full-scale IQ was significantly lower in patients with HLHS (97, P < .001) and patients with UVH (112, P = .024) compared with controls (121). Major neurodevelopmental impairment was found in 26% of the patients with HLHS and 23% of those with UVH, and minor neurologic dysfunction was found in 43% and 46%, respectively. MRI revealed abnormalities, mostly ischemic changes of different degrees, in 82% of the patients with HLHS and in 56% of those with UVH. Prominent changes were significantly associated with neurodevelopmental findings and parental reports of adaptive behavior. In linear regression, significant risk factors for a worse outcome were a history of clinical seizures in connection with the primary operation, a lower diameter of the neonatal ascending aorta, and several pre-, peri-, and postoperative factors related to the primary and bidirectional Glenn operations.
Conclusions:
Although median cognitive performance was within the normal range, neurodevelopmental and brain MRI abnormalities were found in the majority of the patients with UVH, and especially in those with HLHS, at preschool age. Both a narrowed ascending aorta and operation-related factors contributed to these findings.
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