diagnosis on neurodevelopmental outcome; the effect of cardiac surgery on the brain; postoperative factors; developmental care in the intensive care unit (ICU) and early intervention; evaluation and management of neurodevelopmental outcome in children and adolescents with congenital heart disease; HRQOL; and longer-term effects of the initial ICU stay; and the effects of living with chronic cardiac disease on the patient and family The impact of genetic syndromes on neurodevelopment as well as specific genetic abnormalities predisposing to both cCHD and neurodevelopmental delay are also briefly discussed (see also Chapters 4 and 77) Neurodevelopmental and Psychosocial Phenotype in Complex Congenital Heart Disease Survivors An estimated 3 per 1000 children are born each year with cCHD.1 cCHD is defined as congenital heart disease that requires surgical or catheter intervention during the neonatal period or infancy For these children, neurodevelopmental disabilities and psychosocial issues are common, affecting at least 50% of the survivors during childhood and adolescence.2 The individual neurodevelopmental and psychosocial deficits or disabilities may occur in a single or a combination of domains, and may be mild or quite debilitating Formal evaluations of preschool and school-aged children born with cCHD demonstrate a pattern of neurodevelopmental sequelae that includes: mild cognitive impairment with reduced intelligence quotient and academic achievement in math and reading; oromotor dysfunction, expressive speech and language delays; impaired visual-spatial and visual-motor skills; executive dysfunction (organization, planning, and task management); reduced working memory; inattention and hyperactivity; and fine and gross motor delays.3 In addition, a disproportionate number of these cCHD survivors have significant psychosocial issues, including4 impaired social interaction and deficits in social cognition; impaired core communication skills and an increased incidence of autism spectrum disorders; increased incidence of psychiatric disorders; and issues with behavioral and emotional functioning (anxiety, depression, posttraumatic stress symptomatology, and attention deficit hyperactivity disorder) These significant neurodevelopmental and psychosocial morbidities may significantly diminish QOL (Box 76.1).4 Box 76.1 Neurodevelopmental and Psychosocial Challenges Occurring With Increased Frequency in Children, Adolescents, and Young Adults Born With Critical Congenital Heart Disease Neurodevelopmental ■ Stroke ■ Seizures ■ Abnormal brain morphology and functional connectivity (MRI) ■ Abnormal brain growth, cerebral atrophy (CT, MRI) ■ CNS hemosiderin deposition (MRI) ■ Cognitive impairment with lower intelligence quotient and academic achievement in math and reading ■ Oromotor dysfunction ■ Delayed gross and fine motor milestones ■ Decreased gross motor strength, agility, and coordination ■ Speech apraxia ■ Problems with visual–spatial and visual–motor integration ■ Inattention and hyperactivity ■ Impaired working memory Psychosocial ■ Impaired social interaction and deficits in social cognition ■ Impaired core communication skills and an increased incidence of autism spectrum disorders ■ Increased incidence of psychiatric disorders ■ Issues with behavioral and emotional functioning: ■ Anxiety ■ Depression ■ Posttraumatic stress symptomatology ■ Attention deficit hyperactivity disorder CNS, Central nervous system; CT, computerized tomography; MRI, magnetic