Survivorship care was associated with increased knowledge of diagnosis, treatment history, and risk of late effects in both cohorts. Knowledge of late effects decreases with time, suggesting the need for additional educational strategies.

Objectives Despite improved survival in children with hypoplastic left heart syndrome (HLHS), significant concern persists regarding their neurodevelopmental (ND) outcomes. Previous studies have identified patient factors, such as prematurity and genetic syndromes, to be associated with worse ND outcomes. However, no consistent relationships have been identified among modifiable management factors, including cardiopulmonary bypass strategies, and ND outcomes after cardiac surgery in infancy. Studies in immature animals, including primates, have demonstrated neurodegeneration and apoptosis in the brain after certain levels and extended durations of anesthetic exposure. Retrospective human studies have also suggested relationships between adverse ND effects and anesthetic exposure. Methods Cumulative minimum alveolar concentration hours (MAC-hrs) of exposure to volatile anesthetic agents (VAA) (desflurane, halothane, isoflurane and sevoflurane) were collected from an anesthetic database and medical record review for 96 patients with HLHS or variants. ND testing was performed between ages 4 and 5 years including full-scale IQ, verbal IQ, performance IQ and processing speed. Four generalized linear modes were hypothesized a priori and tested using a Gaussian (normal) distribution with an identity link. Results Cumulative VAA exposure ranged from 0 to 35.3 MAC-hrs (median 7.5 hrs). Using specified covariates identified previously as significant predictors of ND outcomes, statistically significant relationships were identified between total MAC-hrs exposure and worse full-scale IQ and verbal IQ scores (p’s < 0.05) alone and after adjusting for relevant covariates. Conclusion Increased cumulative MAC-hrs exposure to VAA is associated with worse ND outcomes in certain domains in children with HLHS and variants.

At time of transplant, pediatric HCT patients frequently have 25-hydroxyvitamin D insufficiency/deficiency, and this occurs more commonly than in the healthy pediatric population. HCT patients rarely follow recommended guidelines to take supplemental vitamin D, consume the Recommended Daily Allowance for vitamin D, or regularly use sunscreen. Further studies are needed to determine whether vitamin D insufficiency/deficiency persists long term in HCT patients and requires dietary and behavioral interventions.

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