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Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: related risk
Anne Sarajuuri1, Eero Jokinen, Riina Puosi
1Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland.
Insights
Children with hypoplastic left heart syndrome and other univentricular heart conditions often experience cognitive and motor deficits. Surgical factors during treatment correlate with these neurological outcomes.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Surgery
Background:
- Children with hypoplastic left heart syndrome (HLHS) and other univentricular heart (UVH) conditions have improved survival but remain at high risk for neurological deficits.
- Cognitive, motor, and other neurologic impairments are common challenges faced by these children.
Purpose of the Study:
- To investigate the prevalence of intellectual and neurological deficits in children with HLHS and UVH.
- To identify potential perioperative and postoperative risk factors contributing to these deficits.
Main Methods:
- A cohort of 27 children with HLHS or UVH, median age 5.70 years, was studied.
- Brain imaging (CT or MRI) was performed on 20 participants.
- Risk factors were correlated with neurodevelopmental outcomes, including IQ and cerebral palsy.
Main Results:
- Patients with HLHS and UVH showed significantly lower mean full-scale IQ scores compared to the general population.
- Abnormal findings on brain imaging, particularly ischemic changes and infarcts, were noted in a subset of patients.
- Diuresis on postoperative day 3 and cardiopulmonary bypass time during the bidirectional Glenn operation were significant predictors of full-scale IQ.
Conclusions:
- Intellectual and neurological deficits are prevalent in children with univentricular heart conditions.
- Perioperative and postoperative factors, specifically related to the primary surgical phase and the bidirectional Glenn operation, significantly contribute to the development of these deficits.
Objective:
Despite improved survival and neurodevelopmental outcome, children with hypoplastic left heart syndrome and other forms of univentricular heart remain at increased risk for cognitive, motor, and other neurologic deficits.
Methods:
We examined 27 children with hypoplastic left heart syndrome or other forms of univentricular heart at a median age of 5.70 years (range 4.99-7.51 years) and performed brain computed tomography or magnetic resonance imaging on 20. Possible risk factors were correlated with outcome.
Results:
Mean full-scale IQ among patients with hypoplastic left heart syndrome was 86.7; that among patients with other forms of univentricular heart was 89.1, with both differing significantly from the expected population mean (P = .015 and P = .029, respectively). Cerebral palsy was diagnosed in 1 of 7 patients with hypoplastic left heart syndrome and 2 of 20 with other forms of univentricular heart. Brain computed tomography or magnetic resonance imaging revealed ischemic changes and infarcts or atrophy in 5 of 8 patients who had undergone the Norwood procedure and in 2 of 12 of those who had not (P = .062). Abnormal computed tomographic findings correlated significantly with lower full-scale IQ (P = .045) and verbal IQ (P = .02). In the multiple linear regression model, diuresis the third day after the primary operation and cardiopulmonary bypass time in the bidirectional Glenn operation correlated significantly with the primary outcome of full-scale IQ.
Conclusion:
In children with univentricular heart, intellectual and neurologic deficits are common. Perioperative and postoperative risk factors related to the primary phase and bidirectional Glenn operation contribute to these deficits.
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