The Neurodevelopmental Outcomes of Patients With Single Ventricles Across the Lifespan.

Abstract

Moderate to severe neurodevelopmental impairments are recognized as a significant comorbidity in one-fourth to one-third of single-ventricle patients. This report reviews the nature of neurodevelopment across the lifespan, contributing factors, and interventions. A focused review was performed of all articles in English that address the topic from 1980 to 2018. Innate patient factors, rather than intraoperative factors, affect the development of neurodevelopmental outcomes, yet a large portion of causality factors remains unexplained. There are also limits to how we currently measure neurodevelopmental outcomes. Limited studies in adulthood and a lack of longitudinal studies hamper our full understanding of how neurodevelopment progresses across time, but estimates are that adults with neurodevelopmental impairments will be affected by abnormal brain aging and early-onset dementia. Intervention services and their efficacy have not been researched in depth and have not been researched across the lifespan. Further research into neurodevelopmental testing that accurately predicts future functioning is needed. The complexity of implementing systematic and effective neurodevelopmental interventions in the busy lives of patients and families, who often have multiple other clinical demands, needs a complex and thoughtful solution within the context of a multidisciplinary care team approach. The role of targeted psychological and educational interventions that decrease maternal worry and improve family functioning should also continue to be explored in patients with a Fontan circulation.