Project Summary
Brain injury and dysfunction in young adults with complex congenital heart disease (CHD): Defining the natural history and potentially modifiable contributors
What is the issue for NSW?
With improvements in care, the number of people living with a Fontan circulation continues to grow worldwide. Neurodevelopmental disability in children with complex CHD is well recognised but is not well-characterised in adults despite high rates of joblessness, carer burden and health services utilisation. We have shown that young people living with a Fontan circulation have neurological injury and neurocognitive impairment that is worse in adults than children. It remains unclear whether this relates to an ‘era effect’ reflecting the evolution of improved perioperative neurological protection or progressive neurological injury related to Fontan physiology. The provision of adequately tailored care to individual’s health needs remains a challenge for survivors due to poorly characterised and complex health problems. A lack of knowledge on neurocognitive function and outcomes is preventing the implementation of targeted strategies, consequently increasing resource utilisation and economic burden on NSW and Australia-New Zealand (ANZ) healthcare systems.
What does the research aim to do and how?
The research aims to determine the natural history and understand the modifiable factors that contribute to brain dysfunction, ultimately to optimise neurocognitive function. A 5-year prospective longitudinal study design will be employed. Demographic data will be retrospectively collected from existing records in the ANZ Fontan Registry. Clinical data will be collected from medical records at treating hospitals. Assessments include neurocognitive testing, quality of life assessment, brain magnetic resonance imaging (MRI) with volumetric analysis, genetic assessment, peripheral venous pressure measurement (resting Fontan pressure), biochemical assessment for markers of inflammation and metabolomic testing. The health economic impact of neurocognitive dysfunction will also be investigated.