Background: Congenital heart disease (CHD) is associated with an increased risk of brain abnormalities. Studies indicate a particular vulnerability of the hippocampus to hypoxia and inflammation. Yet, information regarding the hippocampus and its relation to cognitive function in school-age children with CHD remains scarce.Methods: Children who underwent cardiopulmonary bypass surgery for CHD (N = 17) and healthy controls (N = 14) at 10 years of age underwent neurodevelopmental assessment and cerebral magnetic resonance imaging to measure IQ, working memory performance and hippocampal volume.Results: IQ was significantly lower in children with CHD compared to controls (98 vs 112,P = 0.02). Children with CHD showed worse working memory performance with significantly lower scores in the letter-number sequencing test (P = 0.02). After adjusting for total brain volume, hippocampal volume was smaller in children with CHD compared to controls (P < 0.01). Smaller hippocampal volume was associated with lower IQ (P = 0.04), and digit span scaled score (P = 0.03), but not with other working memory tests (P > 0.1).Conclusion: This study suggests that the hippocampus may be particularly susceptible in children with CHD thereby contributing to cognitive impairments. Further research is necessary to understand the contribution of the hippocampus to cognitive impairments in children with CHD.Impact: IQ is significantly lower in school-age children with congenital heart disease compared to controls. Working memory performance seems to be worse in children with congenital heart disease. Smaller hippocampal volume is associated with lower IQ and seems to be associated with lower working memory performance. The study adds knowledge on the etiology of cognitive impairments in school-age children with congenital heart disease.

Children with complex congenital heart disease (cCHD) are at risk for neurodevelopmental impairments (NDI). This study aims to examine the proportion of school-age children with cCHD who exhibit no NDI and to identify protective factors. Outcomes of five neurodevelopmental domains were assessed longitudinally in children with cCHD. For this retrospective analysis of prospectively collected data, survival without impairments was defined as scores within 1 SD or above 10^th percentile, for each domain. Regression analyses were conducted to identify protective factors and to investigate associations between number of impaired domains and the need for therapies and educational support. Mean outcomes were within the normal range except for motor skills (z = -1.5, SD = 1.6). At age 10, 77.9% of 113 children showed impairments in at least one domain; only 22.1% had none. Having impairments in more domains was associated with higher rates of educational support (p = 0.04). Higher socioeconomic status (SES) (p = 0.02) and motor (p = 0.004) scores at 4 years predicted better outcomes. Despite mostly normal average outcomes, only one quarter of children with cCHD remain free of NDI by age 10. Higher SES and early motor functions predict survival without impairments, highlighting the importance of ongoing monitoring and intervention. Only one quarter of children born with complex congenital heart disease remain free of neurodevelopmental impairments at school age. 42.5% of school-age children with complex CHD show neurodevelopmental impairments in multiple domains, highlighting the need for ongoing monitoring and intervention to improve outcome. Socioeconomic status and early motor outcomes predict later survival without neurodevelopmental impairments.

Background/Objectives: Hypoxic-ischemic encephalopathy (HIE) in late preterm and term neonates accounts for neonatal mortality and unfavorable neurodevelopmental outcomes in survivors despite therapeutic hypothermia (TH) for neuroprotection. The circumstances of death in neonates with HIE, including involvement of neonatal palliative care (NPC) specialists and neurodevelopmental follow-up at 18–24 months in survivors, warrant further evaluation. Methods: A retrospective multicenter cohort study including neonates ≥ 35 weeks gestational age with moderate to severe HIE receiving TH, registered in the Swiss National Asphyxia and Cooling Register between 2011 and 2021. Neurodevelopmental follow-up at 18–24 months in survivors was assessed. The groups of survivors and deaths were compared regarding perinatal demographic and HIE data. Prognostic factors leading to redirection of care (ROC) were depicted. Results: A total of 137 neonates were included, with 23 (16.8%) deaths and 114 (83.2%) survivors. All but one death (95.7%) occurred after ROC, with death on a median of 3.5 (2–6) days of life. Severe encephalopathy was indicated by a Sarnat score of 3 on admission, seizures were more frequent, and blood lactate values were higher on postnatal days 1 to 4 in neonates who died. Lactate in worst blood gas analysis (unit-adjusted odds ratio 1.25, 95% CI 1.02–1.54, p = 0.0352) was the only variable independently associated with ROC. NPC specialists were involved in one case. Of 114 survivors, 88 (77.2%) had neurodevelopmental assessments, and 21 (23.9%) of those had unfavorable outcomes (moderate to severe disability). Conclusions: Death in neonates with moderate to severe HIE receiving TH almost exclusively occurred after ROC. Parents thus had to make critical decisions and accompany their neonate at end-of-life within the first week of life. Involvement of NPC specialists is encouraged in ROC so that there is continuity of care for the families whether the neonate survives or not.

Background/Objectives: Hypoxic-ischemic encephalopathy (HIE) in (near)term neonates accounts for neonatal mortality and unfavorable neurodevelopmental outcome in survivors, despite therapeutic hypothermia (TH) for neuroprotection. The circumstances of death in neonates with HIE and involvement of neonatal palliative care (NPC) specialists warrant further evaluation. Methods: Retrospective multicenter cohort study including neonates ≥ 35 weeks gestational age with moderate to severe HIE receiving TH, registered in the Swiss National Asphyxia and Cooling Register between 2011 and 2021. Neurodevelopmental follow-up at 18-24 months in survivors was assessed. The groups of survivors and deaths were compared regarding perinatal demographic and HIE data. Prognostic factors leading to redirection of care (ROC) were depicted. Results: 137 neonates were included, 23 (16.8%) deaths and 114 (83.2%) survivors. All but one death (95.7%) occurred after ROC with death on median 3.5 (2-6) days of life. Severe encephalopathy indicated by Sarnat score 3 on admission and seizures were more frequent, and blood lactate values were higher on postnatal days 1 to 4 in neonates who died. Lactate in worst blood gas analysis (unit aOR 1.25, 95% CI 1.02-1.54, p=0.0352) was the only variable independently associated with ROC. NPC specialists were involved in one case. Of 114 survivors, 88 (77.2%) had neurodevelopmental assessments, and 21 (23.9%) of those had unfavorable outcome (moderate to severe disability). Conclusions: Death in neonates with moderate to severe HIE receiving TH almost exclusively occurred after ROC. Parents thus had to take critical decisions and accompany their neonate at end-of-life within the first week of life. To ensure continuity of care for the family, also beyond the death of the neonate, involvement of NPC specialists is encouraged in ROC.