Strategies for the Evaluation and Management of Neurodevelopmental Outcomes in Children and Adolescents With Congenital Heart Disease In 2012, the American Heart Association (AHA) published a comprehensive scientific statement formally identifying and stratifying CHD survivors at risk for worse neurodevelopmental outcome, outlining a surveillance, screening, evaluation, and management algorithm for CHD survivors, and creating recommendations to optimize neurodevelopmental outcome in the pediatric CHD population.3,171 This statement was also formally endorsed by the American Academy of Pediatrics A CHD-specific neurodevelopmental algorithm was constructed to supplement the 2006 Academy of Pediatrics statement on developmental surveillance and screening.3 It is intended that the algorithm be carried out within the context of the medical home Developmental disorders can be identified and managed through surveillance, screening, early evaluation, periodic reevaluation, and continuous, comprehensive treatment coordinated through the medical home The child's primary pediatrician, pediatric cardiologist, psychologist or developmental-behavioral pediatrician may lead care coordination Children with significant difficulties often benefit from a multidisciplinary treatment approach, including special education classes, tutoring, behavior management counseling, and physical, occupational, and speech/language therapies The management algorithm stratified children with CHD for neurodevelopmental outcome based on established risk factors.3 Box 76.4 delineates the categories of CHD patients considered high-risk for developmental disorders or disabilities and the specific recommendations made by the AHA.3 For those deemed to be at high-risk for developmental deficits or developmental delay, formal, periodic developmental, and medical evaluations are recommended to assess neurodevelopmental, psychosocial, behavioral, and emotional functioning Other recommendations include: (1) refer high-risk children for early intervention even before a developmental disorder is diagnosed; (2) reevaluate for developmental disorders and developmental delays periodically in children with CHD deemed high-risk at 12 to 24 months, 3 to 5 years, and 11 to 12 years of age; and (3) consider counselling high-risk children for educational or vocational options when they reach young adulthood If potential developmental problems can be identified earlier, the hope is to prevent issues from developing in school that will impede children with CHD from reaching their full potential These additional recommendations were supported by research conducted by Mussatto et al in a longitudinal study testing for developmental issues in CHD surgical survivors in early childhood They found that exposure to risk and the prevalence of delay changes over time, warranting repeated evaluation in this high-risk population.172 The implementation of these new guidelines allows clinicians to identify patients with important neurodevelopmental issues that may impact HRQOL Box 76.4 Patients at High Risk for Developmental Disorders or Disabilities ■ Neonates or infants requiring open heart surgery ■ Children with other cyanotic heart lesions not requiring open heart surgery during the neonatal or infant period ■ Any combination of CHD and the following comorbidities: prematurity (2 weeks, perioperative seizures, significant abnormalities on neuroimaging, microcephaly CHD, Congenital heart disease; ECMO, extracorporeal membrane oxygenation; VAD, ventricular assist device From Marino BS, Lipkin PH, Newburger JW, et al Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association Circulation 2012;126:1143–1172 If a child fits high-risk criteria, it is recommended that the “medical home” schedule evaluations to assess neurodevelopmental, psychosocial, and behavioral and emotional functioning (see also Chapter 85) The child's cardiologist should continue to manage the cardiovascular issues related to the CHD, but other medical providers and therapists need to join the child's care team The medical home leader, usually the child's primary care physician, will coordinate care and provide the family with an overall approach to managing their child's neurodevelopmental, psychosocial, and physical health needs If potential developmental problems can be identified earlier, the hope is to prevent issues from developing in school that will impede children with CHD from reaching their full potential Cardiac Neurodevelopmental Programs Research supports the benefit of early evaluation and ongoing treatment of developmental issues To provide coordinated care, many pediatric cardiovascular centers have now established multidisciplinary cardiac neurodevelopmental follow-up programs to evaluate, diagnose, and monitor developmental, learning, and behavioral problems.158,173–175 Teams often include developmental-behavioral pediatricians, psychologists, educators, occupational therapists, physical therapists, speech pathologists, neurologists, cardiovascular geneticists, and pediatric cardiologists.158–159 Educators can encourage families to share the results of multidisciplinary evaluations with the child's school system to ensure that recommendations are implemented in the school setting Providers who care for a child with CHD are encouraged to talk to the medical home leader, if present, about resources in their medical center and/or community to screen, evaluate, and periodically reevaluate throughout childhood to enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.3 Developmental and School Services for Children and Adolescents Children and adolescents with CHD and a known developmental delay or associated genetic anomaly should continue to receive ongoing developmental evaluations and care The types of delays seen in children with CHD are ... include developmental-behavioral pediatricians, psychologists, educators, occupational therapists, physical therapists, speech pathologists, neurologists, cardiovascular geneticists, and pediatric cardiologists.158–159 Educators can... Research supports the benefit of early evaluation and ongoing treatment of developmental issues To provide coordinated care, many pediatric cardiovascular centers have now established multidisciplinary cardiac neurodevelopmental follow-up programs to evaluate, diagnose, and monitor