Developmental Disabilities Create Academic Challenges for Kids with Complex CHD

As mortality rates for children with congenital heart disease (CHD) have steadily fallen and even children with complex CHD are likely to live to adulthood, clinicians and researchers have turned their attention to the assessment of long-term morbidities with the hope of developing effective treatment strategies and, ultimately, preventing morbidity entirely. Neurodevelopmental morbidities have been identified as a significant issue for children with complex CHD and can have a negative impact on early childhood development, academic performance, and later transition to adulthood.¹ A pattern of developmental impairments characterized by mild cognitive impairment, impaired social interaction, and impairments in core communication skills including pragmatic language, as well as, inattention, impulsive behavior, and impaired executive function have been identified in patients with complex CHD.^2-4 Children with CHD are at greater risk for developmental disabilities (DD) due to biologic factors such as abnormal cardiac physiology and the surgical and medical therapies used to treat their heart disease or underlying genetic disorders. Environmental factors such as family environment, parenting styles, socioeconomic factors, and school and work environments can be protective or can increase risk. For many children, early assessment and the assistance of specialized educational or rehabilitation services (speech, physical therapy, and occupational therapy) can improve their developmental outcomes.

Identifying children with CHD who are at higher risk for DD is an important first step, raising awareness among parents and caregivers to the potential for developmental and behavioral impairments throughout childhood. Hopefully, this will lead to prompt assessment and timely referral for special services. This scientific statement ably addresses this issue. The authors have synthesized the current research and proposed an algorithm of risk stratification into high- and low-risk groups. Recognizing the wide range of congenital heart defects that require very different treatment modalities with distinct risk profiles, some children are clearly at greater risk for neurodevelopmental morbidity than other children. The high-risk group includes patients with complex CHD that require open heart surgery early in infancy and those with other cyanotic heart lesions that may not require infant surgery. Important co-morbidities that increase the risk of DD include prematurity, genetic syndromes, history of mechanical support or periods of CPR, heart transplantation, and those with perioperative seizures associated with CHD surgery. Prolonged hospital stay greater than 2 weeks, developmental delays identified in infancy, the presence of microcephaly, and significant abnormalities seen on neuroimaging are also associated with increased risk.¹

Beyond identifying children at higher risk, the scientific statement presents a framework for surveillance, screening, and evaluation of DD that serves as a supplement to the 2006 American Academy of Pediatrics (AAP) policy statement, “Identifying Infants and Young Children with Developmental Disorders in the Medical Home: An Algorithm for Developmental Surveillance and Screening.”⁵ In keeping with recommendations from the AAP on developmental screening for all children, the medical home is identified as the site of primary screening for developmental disorders and coordination of care with specialty providers.⁵ This model allows for an individual approach to each child in their local community, the maintenance of a comprehensive health record, and close coordination and collaboration with pediatric sub-specialists, therapists, and educators.

There are a variety of instruments for developmental screening available for use in the outpatient setting that employ parent questionnaires or direct patient testing. They are designed for use with certain age groups and assess specific aspects of development. Formal screening for all children is recommended at 9, 18, and 30 months.5 Additional screening at the 4-year-old visit is recommended for children with CHD to assess for school readiness and behavior issues.¹ Autism spectrum disorders are more prevalent among high-risk children with CHD than in the general population,^6-8 and specific screening is recommended at 18 and 24 months. If developmental deficits are identified on routine screening, infants and young children are referred for early intervention services in their community or for more formal developmental testing. Many infants with complex heart disease are referred to early intervention programs at the time of discharge from the hospital. While low-risk children with CHD can be well-served by the screening and surveillance for DD and autism spectrum disorders recommended by the AAP,⁵ children at high risk for DD should undergo formal developmental evaluation.¹

Some DD in children with CHD may not be apparent until school-age or adolescence. Longitudinal studies by Hovels-Gurich and colleagues^9-11 and Newburger and colleagues^3,4,12,13 have identified behavioral issues, specifically in areas of attention, and impairments in social cognition, speech and language, and motor skills in school-age children that were not evident at younger ages. Vulnerabilities may become apparent during times of transition when the complexity of developmental tasks increases. Learning to read at ages 6-7 and reading to learn at ages 8-10 are examples of transition periods. Learning disabilities, behavioral issues, and attention deficit hyperactivity disorder (ADHD) can lead to poor academic performance. Because these issues may present at any point in childhood or adolescence, a higher index of suspicion throughout the school years is needed, and ongoing screening and reevaluation are particularly important for children with complex CHD.

For school-age children and adolescents, evaluation and treatment of DD is primarily based in the school system. Local resources for developmental and behavioral evaluations and special education services can vary widely among different communities and are often overburdened and under-funded. Knowledge of the neurodevelopmental impairments common to children with complex CHD may be lacking in the school system. Increased collaboration between the medical home, pediatric cardiologists, and educators is critical to meet the needs of these children. The health care system and the educational system do not have a tradition of collaboration, and current professional and logistical challenges that create barriers to cooperation need to be addressed. This is important for children with complex CHD as well as other children with chronic health problems who are at risk for DD.

Caregivers in pediatric cardiology should incorporate information on neurodevelopmental morbidities in their daily practice and supplement the screening and evaluation being done in the medical home. Health care systems may need to augment the evaluation resources available within the school with neuropsychological testing and other highly specialized developmental evaluations. Clinicians need to partner with parents and school personnel during the individualized educational plan (IEP) process to provide a complete picture of the child’s needs and work with the school to provide services. Specialized multidisciplinary cardiac neurodevelopmental clinics have been established in some cardiac centers and are a valuable resource to patients, families, and local school systems. They can provide specialized testing and subspecialty assessments and make recommendations for management strategies. They may also play a role in future research and evaluation of treatment options.

The data are clear that our most complex patients are at risk for neurodevelopmental morbidities and these can have a profound effect on academic performance and quality of life. This scientific statement makes a significant contribution by identifying high- risk children with CHD and presenting a framework for developmental screening within the medical home. Reflecting the current research in the field, there are few specifics regarding management strategies. Going forward, pediatric cardiology caregivers, educators, and therapists are challenged to develop effective treatment strategies to support children with known DD so they can reach their full potential. Further attention to potential changes in clinical management that might be more protective of the developing brain is also warranted. The goal for all who work with children with congenital heart disease is a reduction in the incidence of neurodevelopmental morbidities and improved developmental outcomes.