Neonatal Brain Injury and Outcomes in Congenital Heart Disease

Study Questions:

What is the relationship between neonatal brain injury and neurodevelopmental outcome in infants with complex congenital heart disease?


A prospective study was performed at two centers. The study involved term newborns with d-transposition of the great arteries (d-TGA) and single ventricle physiology. Patients underwent preoperative and postoperative brain magnetic resonance imaging (MRI) and neurodevelopmental outcome assessment at 12 and 30 months with the Bayley Scales of Infant Development-II. Brain injury was categorized by the brain injury severity score and white matter injury (WMI) was quantified by volumetric analysis.


A total of 165 neonates were enrolled, of which 33 patients were lost to follow-up and 28 patients died prior to the 12-month follow-up, leaving 104 patients present for the 12-month follow-up. By the time of the 30-month follow-up, an additional 32 patients had been lost to follow-up and 2 had died, leaving 70 patients for the 30-month follow-up. At 12 months, only clinical variables and not imaging characteristics were associated with neurodevelopmental outcome. At 30 months, subjects with moderate-to-severe WMI had significantly lower Psychomotor Development Index scores (13 points lower) as compared with no or minimal WMI after adjusting for various factors. Stroke was not associated with outcome.


The authors concluded that an increasing burden of WMI is associated with worse motor outcomes for infants with critical congenital heart disease. No adverse association was seen between small strokes and outcome.


This study investigated the relationship between perioperative brain MRI findings and neurodevelopmental outcomes in infants with d-TGA and single ventricle congenital heart disease. The study supports the prognostic value of perioperative brain imaging as well as the need to perform continued neurodevelopmental assessments beyond 1 year of age. Interestingly, this study also assessed the controversial area of the relationship between balloon atrial septostomy (BAS) and neurologic injury. The study showed a significant relationship between the need for BAS and preoperative brain injury, especially stroke. The vast majority of strokes (86%) were small, and paradoxically, BAS and stroke were associated with better neurodevelopmental outcome on the univariable analysis. The authors speculate that the impact of BAS on improving oxygen delivery may improve neurodevelopmental outcomes. This area requires additional study.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Quality Improvement, Magnetic Resonance Imaging

Keywords: Brain Injuries, Diagnostic Imaging, Infant, Newborn, Heart Defects, Congenital, Injury Severity Score, Magnetic Resonance Imaging, Neuroimaging, Neurology, Pediatrics, Stroke, Transposition of Great Vessels, Term Birth

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