Congenital heart disease: A primer for the pediatric neuropsychologist

Abstract

Congenital heart disease (CHD) affects millions of people worldwide, including over one million children in the United States. Approximately 25% of children born with CHD require intensive surgical intervention within the first year of life. Despite improved rates of survival into adulthood - rates that exceed 90% in the modern era - children and adolescents with CHD remain at risk for neurological injury and a range of neurobehavioral and psychosocial challenges that pose a threat to quality of life across the lifespan. Consequently, as experts in both clinical psychology and brain development, neuropsychologists are becoming increasingly involved in cardiac follow-up and monitoring to promote optimal developmental outcomes. The primary objective of this paper is to provide an evidence-based, clinically-oriented primer on CHD for pediatric neuropsychologists working with this growing population of survivors. Following an introduction to current standard-of-care guidelines for managing children and adolescents with CHD, we present an overview of brain development within the context of CHD, review neuropsychological outcomes, examine factors influencing variability in outcomes, and discuss implications and strategies for clinical assessment.

Child neurodevelopment and mental health after surgical ventricular septal defect repair: risk and protective factors

“…Socio-economic status was calculated as a sum index of maternal and paternal secondary education level (four stages: <9 [1], 9 [2], 10-12 [3], or 13 [4]y of education), maternal and paternal origin (two stages: international [0] or national [1]), and monthly family income (six stages: less than €1000 [1], €1000-€2000 [2], €2000-€3000 [3], €3000-€4000 [4], €4000-€5000 [5], more than €5000 [6]) with a theoretical range of 3 to 16.…”

“…Ventricular septal defects (VSDs) are the most common congenital heart defects and constitute approximately 30% of all congenital heart diseases (CHDs) . Generally, most long‐term studies on CHDs show that affected children are developmentally impaired . It is accepted that the severity of the impairment varies with the complexity of CHD .…”

“…1 Generally, most long-term studies on CHDs show that affected children are developmentally impaired. 2,3 It is accepted that the severity of the impairment varies with the complexity of CHD. 4,5 Studies on more severe CHD show that a longer stay in the intensive care unit or total hospital stay 6 and a higher age at surgery 7 predicted developmental and emotional or behavioural impairments.…”

Cognitive flexibility in critical CHD: a target for intervention

“…3 Indeed, it is now understood that brain development within the context of critical CHD (i.e., CHDs requiring intensive surgical intervention(s) to sustain life) is often atypical, with signs of deviation from expected neuronal maturation and volumetric trajectories recognisable as early as the late-second/early-third prenatal trimester [4][5][6][7][8] and evidence of structural, functional, and network topological brain abnormalities documented throughout adolescence and young adulthood. [9][10][11][12][13][14][15][16][17][18][19][20] Children and adolescents with critical CHD are also at heightened risk for a range of neurobehavioural and psychosocial challenges, 21 most notably in executive function. [22][23][24][25] Executive function is an umbrella term, encompassing three core components: inhibitory control, working memory, and cognitive flexibility.…”

EPAS 1, congenital heart disease, and high altitude: disclosures by genetics, bioinformatics, and experimental embryology

“…Congenital heart disease (CHD) affects millions of individuals worldwide, including over one million children in the United States with about one-fourth of children born with CHD requiring intensive surgical intervention within the first year of life. Despite improved cardiac surgical procedures and rates of survival into adulthood, incidences that surpass 90% of children remain at risk for neurological injury and neurobehavioral challenges that pose a threat to the quality of life across the lifespan [1]. The high-altitude environment is a challenge for human settlement.…”