Premature birth and congenital heart disease are two of the most common conditions leading to hospital stay for newborns in developed countries. Both conditions are associated with altered brain development and risk of brain injury. Our goal is to understand the best approach for treating heart defects to optimize neurodevelopment in babies born early with heart conditions.

This two site study is being carried out at UCSF Benioff Children's Hospital San Francisco and The Sick Children's Hospital in Toronto.  The study seeks to enroll 225 subjects born at a gestational age < 37 weeks with critical congenital heart disease requiring surgery before 48 weeks corrected gestational age.   

Preterm birth and congenital heart disease (CHD) are two of the most common sources of perinatal morbidity in high resource countries.  Both conditions are associated with acquired brain injury and adverse neurodevelopmental outcomes.  Very little is known about the combined risk for newborns with congenital heart disease that are born preterm and how this risk is affected by variable approaches to palliative or definitive surgical strategies to repair heart defects.  In addition to brain injury, an increasing number of genetic anomalies have been identified in CHD that may contribute to ND outcomes.   Our extensive experience imaging term babies with CHD and preterm babies without CHD, combined with high and increasing volume of this vulnerable population at our two centers uniquely positions us to describe the risk and magnitude of acquired brain injury as well as comparative brain development in CHD newborns born preterm with respect to intervention strategies.  Our long-term goal is to optimize neurodevelopmental outcomes. In this proposal, we will leverage cross-center practice variability in timing and choice of surgical interventions, palliative versus definitive, to determine the association of surgical intervention strategy with brain development and risk of brain injury. We will also perform a comprehensive genetic evaluation to determine how genetic anomalies alter susceptibility to brain injury and ND outcome.

White matter injury (WMI) Topography Reflects White Matter Maturation: Comparison of the WMI map of 86 term CHD neonates and that of 58 preterm newborns scanned early-in-life overlaid on the T1-weighted term brain template. WMI probability in term CHD (1st column) and premature newborns is color coded according to the scale at left.  In the third column, WMI distribution is shown according to only premature (blue), only CHD (magenta) and common location (white).  WMI topology can be seen as more central in premature newborns at early scan. Data from Guo et al., Neuroimage 2019 185(15)742-749.