Neurodevelopmental outcomes in children with congenital heart disease

By Cheryl L. Brosig, PhD

Congenital heart disease is the No. 1 birth defect in the U.S., affecting approximately 1 in every 100 births or about 40,000 babies per year. As surgical advances have been made, mortality rates have declined significantly, even for those children born with the most severe heart lesions. Because children with congenital heart disease are living longer, there has been more emphasis placed on outcomes beyond mere survival. Parents of these children often ask: "What will my child be like when he or she gets to kindergarten?" "Will my child be normal?" It only has been in recent years that research has explored the neurodevelopmental outcomes for these children.

Children with congenital heart disease are at risk for neurodevelopmental problems due to things that can occur before these children have surgery, during surgery or after surgery. Following are some preoperative risk factors:

• Congenital brain anomalies.
• Genetic syndromes, such as Down syndrome or 22q11 deletion syndrome.
• Poor perfusion.
• Shock and/or acid/base disturbances.
• Hypoxia.
• Failure to thrive.
• Blood clots.

During surgery, other risk factors may occur, such as:

• Seizures.
• Cardiopulmonary bypass (inflammatory response; risk of blood clots or air entry).         
• Deep hypothermic circulatory arrest (cooling time; acid-base management strategy).
• In the early postoperative period, risk factors include:
• Low cardiac output/poor perfusion.
• Hypoxia.
• Seizures.
• Laboratory disturbances.
• Excessive bleeding or blood clotting.
• Infection.

On a long-term basis, there are other psychosocial factors that may have a negative impact on these children, including:

• Physical restriction.
• Parental overprotection.
• School absence.
• Decreased peer interactions.

All of these issues place children with congenital heart disease at increased risk for neurodevelopmental and psychosocial problems.

Research suggests that the more risk factors the child encounters, the greater the likelihood that he or she will have neurodevelopmental problems. Although severe neurological impairment in these children is rare, and most of the children have normal intelligence, a significant number of these children have problems in the following areas:

• Higher-order language skills.
• Motor skills.
• Attention span/hyperactivity.
• Visual-spatial functioning.

From an emotional standpoint, research suggests that most children with congenital heart disease and their families are coping well and report positive quality of life, however, there are a number of children who exhibit behavioral problems, anxiety or depression.  In addition, some parents report high levels of stress related to the impact the child's illness has on the family.

Intervention

For practitioners working with these children and families, it is important to ask about the child's developmental progress. Infants and children younger than 3 years who exhibit developmental delays may be eligible for physical, occupational and/or speech therapy services through a Birth-to-Three Program. Children from 3 to 5 years old may be eligible for early childhood services through the public school system. For school-age children, parents can request an evaluation conducted by a multidisciplinary team at the child's school to determine whether the child needs special education services as part of an Individualized Education Plan. It is possible that the child will be able to receive services under a 504 Plan, also known as Other Health Impaired, using their cardiac diagnosis as their qualifying medical condition.

In addition to asking about the child's developmental and academic progress, practitioners need to ask about the child's behavioral/emotional functioning.  Furthermore, practitioners should ask about how the family is functioning in general.  Some of the following questions may be helpful:

• Is the child having any behavioral/emotional problems at home?
• Is the child having any behavioral/emotional problems at school?
• Does the child have friends?
• How is the stress level within the family?
• Is the child's illness having any negative impact on the family?

Based on answers to these questions, practitioners then can determine whether referral to a pediatric psychologist or other mental health practitioner may be needed.

Although many children with congenital heart disease do well from a neurodevelopmental/psychological standpoint, there are some children who have problems in these areas. Because these children and families may not have frequent contact with their cardiologist, especially after they are surgically repaired, it is extremely important for general practitioners, who have more frequent contact with these children, to screen for neurodevelopmental and psychosocial problems.

Cheryl Brosig, PhD, is a pediatric psychologist at Children's Hospital of Wisconsin. She also is an associate professor of Pediatrics (Cardiology) at the Medical College of Wisconsin and a member of Children's Specialty Group.

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