Neurodevelopmental Screening The decrease in mortality seen in survivors of CHD has allowed clinicians to begin to shift their focus toward minimizing morbidity and optimizing long-term neurodevelopmental and psychosocial outcomes Children with CHD are a heterogenous group with a wide spectrum of disease burden, but it is clear that even children with just a single exposure to cardiopulmonary bypass may require rehabilitative services in childhood.133 Many children with CHD also have additional risk factors for developmental delay such as prematurity, underlying genetic conditions, and exposure to mechanical circulatory support or cardiopulmonary resuscitation Much of the framework for the developmental challenges faced by such children has been extrapolated from 2 decades worth of follow-up provided by the Boston Circulatory Arrest Study (BCAS) cohort.134 The most recent follow-up of these children in both elementary and high school was notable for below-average performance on tests of memory, executive functioning, visual spatial skills, attention, and social cognition despite IQ testing, with approximately 65% of the cohort requiring remedial academic and/or behavioral sciences by adolescence.135,136 The developmental profile noted in CHD survivors133 is characterized by: ■ Mild cognitive delays and reduced school performance ■ Neuropsychological issues in executive functioning, visual-spatial skills, and working memory ■ Behavioral difficulties including inattention and increased impulsivity ■ Difficulty with communication and social skills ■ Mild fine- and gross-motor delays Because these children may manifest mild delays in multiple domains, the impact of their deficits is often underappreciated Mild delays in multiple areas may go unrecognized without formal testing This can result in delayed implementation of services that might improve developmental, functional, and academic outcomes As early developmental intervention has been shown to improve outcomes in other high-risk populations,137,138 timely identification/screening for neurodevelopmental and psychosocial problems and focused intervention coordinated by the medical home is paramount The American Heart Association currently recommends early evaluation/screening and treatment for three categories of pediatric CHD patients known to be at high risk for developmental disability (Box 89.1) who should receive early evaluation and treatment.133 Pediatricians and cardiologists must work together to ensure that high-risk children with CHD are being referred for a comprehensive neurodevelopmental evaluation, but also recognize findings within each developmental domain that should trigger a more in-depth evaluation in children who would otherwise be considered lower risk Box 89.1 Categories of High-Risk Patients Who Require Developmental Screening/Referral Survivors of neonatal or infant open heart surgerya (palliative or corrective) Children with cyanotic heart disease who do not require open heart surgery in infancyb Children with congenital heart disease AND other high-risk comorbidities: ■ Prematurity (GA 14 days) CPR, Cardiopulmonary resuscitation; ECMO, extracorporeal membrane oxygenation; GA, gestational age; LOS, length of stay; VAD, ventricular assist device aExamples of congenital heart disease that may require infant open heart surgery include: double-inlet left ventricle, double-outlet right ventricle, hypoplastic left heart syndrome, interrupted aortic arch, pulmonary atresia with intact ventricular septum, tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, total anomalous pulmonary venous connection bExamples of cyanotic heart disease include tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals, Ebstein anomaly The current algorithm recommends that high-risk children should receive direct referral for a formal multidisciplinary developmental evaluation and Early Intervention (Fig 89.2) A formal developmental assessment should be repeated at 12 to 24 months, 3 to 5 years, and 11 to 12 years of age to reassess and treat ongoing neurodevelopmental and psychosocial issues or identify latent issues that were not previously appreciated Children identified as low risk should undergo heightened surveillance and screening according to the general American Association of Pediatrics guidelines, including the standard developmental screening at 9, 18, 30, and 48 months in addition to autismspecific screening at 18 to 24 months of age.139 ... and treatment for three categories of pediatric CHD patients known to be at high risk for developmental disability (Box 89.1) who should receive early evaluation and treatment.133 Pediatricians and cardiologists must work together to ensure... undergo heightened surveillance and screening according to the general American Association of Pediatrics guidelines, including the standard developmental screening at 9, 18, 30, and 48 months in addition to autismspecific screening at 18 to 24 months of age.139