Developmental Disabilities Create Academic Challenges for Kids with Complex CHD

Updated:Aug 5,2014

Disclosure: Ms. O’Brien has nothing to disclose.
Pub Date: Monday, July 30, 2012
Author: Patricia O’Brien, RN, MSN, CPNP-AC
Affiliation: Department of Nursing/Patient Services, Cardiovascular Program, Children’s Hospital, Boston, Mass.

Article Text

Citation: Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson WH Jr, Li J, Smith SE, Bellinger DC, Mahle WT; on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular and Stroke Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012: published online before print July 30, 2012, 10.1161/CIR.0b013e318265ee8a. 

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 colleagues9-11 and Newburger and colleagues3,4,12,13 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.


  1. Marino, BS, Lipkin PH, Newburger JW, et al.  Neurodevelopmental outcomes in children with congenital heart effects: Evaluation and management.  A Scientific Statement of the American Heart Association, 2012
  2. Gaynor JW, Nord AS, Wernovsky G, Bernbaum J, Solot CB, Burnham N, Zackai E, Heagerty PJ, Clancy RR, Nicolson SC, Jarvik GP, Gerdes M. Apolipoprotein e genotype modifies the risk of behavior problems after infant cardiac surgery. Pediatrics. 2009;124:241-250. 
  3. Bellinger DC, Newburger JW, Wypij D, Kuban KC, duPlessis AJ, Rappaport LA. Behaviour at eight years in children with surgically corrected transposition: The Boston circulatory arrest trial. Cardiol Young. 2009;19:86-97.
  4. Bellinger DC, Wypij D, duPlessis AJ, Rappaport LA, Jonas RA, Wernovsky G, Newburger JW. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: The Boston circulatory arrest trial. J Thorac Cardiovasc Surg 2003;126:1385-1396.
  5. Identifying infants and young children with developmental disorders in the medical home: An algorithm for developmental surveillance and screening. Pediatrics. 2006;118:405-420.
  6. Antshel KM, Aneja A, Strunge L, Peebles J, Fremont WP, Stallone K, Abdulsabur N, Higgins AM, Shprintzen RJ, Kates WR. Autistic spectrum disorders in velo-cardio facial syndrome (22q11.2 deletion). J Autism Dev Disord. 2007;37:1776-1786.
  7. Wier ML, Yoshida CK, Odouli R, Grether JK, Croen LA. Congenital anomalies associated with autism spectrum disorders. Dev Med Child Neurol. 2006;48:500-507.
  8. Hultman CM, Sparen P, Cnattingius S. Perinatal risk factors for infantile autism. Epidemiology. 2002;13:417-423.
  9. Hovels-Gurich HH, Konrad K, Skorzenski D, Herpertz-Dahlmann B, Messmer BJ, Seghaye MC. Attentional dysfunction in children after corrective cardiac surgery in infancy. Ann Thorac Surg. 2007;83:1425-1430.
  10. Hovels-Gurich H, Konrad K, Wiesner M, Minkenberg R, Herpertz-Dahlmann B, Messmer B, Von Bernuth G. Long term behavioural outcome after neonatal arterial switch operation for transposition of the great arteries. Arch Dis Child. 2002;87:506 – 510.
  11. Hovels-Gurich HH, Seghaye MC, Schnitker R, Wiesner M, Huber W, Minkenberg R, Kotlarek F, Messmer BJ, Von Bernuth G. Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation. J Thorac Cardiovasc Surg. 2002;124:448-458.
  12. Bellinger DC, Wypij D, Kuban KC, Rappaport LA, Hickey PR, Wernovsky G, Jonas RA, Newburger JW. Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. Circulation. 1999;100:526-532.
  13. Bellinger DC, Wypij D, Rivkin MJ, DeMaso DR, Robertson RL, Dunbar-Masterson C, Rappaport LA, Wernovsky G, Jonas RA, Newburger JW. Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure/clinical perspective. Circulation. 2011;124:1361-1369.
-- The opinions expressed in this commentary are not necessarily those of the editors or of the American Heart Association --

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