Optimizing Neurodevelopmental Outcomes in Neonates With Congenital Heart Disease

Abstract

Neurodevelopmental impairment is a common and important long-term morbidity among infants with congenital heart disease (CHD). More than half of those with complex CHD will demonstrate some form of neurodevelopmental, neurocognitive, and/or psychosocial dysfunction requiring specialized care and impacting long-term quality of life. Preventing brain injury and treating long-term neurologic sequelae in this high-risk clinical population is imperative for improving neurodevelopmental and psychosocial outcomes. Thus, cardiac neurodevelopmental care is now at the forefront of clinical and research efforts. Initial research primarily focused on neurocritical care and operative strategies to mitigate brain injury. As the field has evolved, investigations have shifted to understanding the prenatal, genetic, and environmental contributions to impaired neurodevelopment. This article summarizes the recent literature detailing the brain abnormalities affecting neurodevelopment in children with CHD, the impact of genetics on neurodevelopmental outcomes, and the best practices for neonatal neurocritical care, focusing on developmental care and parental support as new areas of importance. A framework is also provided for the infrastructure and resources needed to support CHD families across the continuum of care settings.

Figure 1.

Risk Factors for Neurological and Neurodevelopmental Abnormalities. Schematic representation of prenatal, perioperative, and social/environmental factors that contribute to neurodevelopmental disabilities in CHD. Prenatally, abnormal cardiac anatomy can alter blood flow and oxygenation to the developing brain, leading to impaired brain development. Concurrently, other pregnancy exposures including abnormal placental development and function and parental well-being can also have a negative impact on the brain. Genetic disruption of brain development may also occur via underlying genetics syndromes or pathogenic variants. Susceptibility genes (i.e., apolipoprotein E ε2) can further contribute to postoperative neurodevelopmental deficits. A variety of physiologic/intensive care exposures, such as hypoxemia, additional surgery, and drug exposure are important predictors in the neurocritical care arena. Finally, home environment and socioeconomic status can have a positive or negative influence, depending on the infant’s environment, whereas developmental care interventions that begin in the intensive care unit and continue after discharge may have neurodevelopmental benefit. CBF, cerebral blood flow; ND, neurodevelopment; WM, white matter. Adapted with permission from J. William Gaynor.

Figure 2.

Timing and Pattern of Neuroimaging Abnormalities. This timeline depicts abnormalities identified across key brain regions in neuroimaging studies of brain development in patients with CHD, beginning during the fetal period and extending into adolescence/adulthood. Representative T2-weighted images are provided for each developmental epoch depicting the significant change in brain size and structure that occurs during this period. As demonstrated, brain abnormalities in CHD are global in nature, involving white and gray matter regions at the micro- and macro-structural level. BPD, biparietal diameter; HC, head circumference; TBV, total brain volume.