The following are key points to remember from an American Heart Association Scientific Statement on neurodevelopmental outcomes for individuals with congenital heart disease, which includes updates in neuroprotection, risk-stratification, evaluation, and management:

1. This scientific statement serves as an update to the 2012 scientific statement and reflects the significant expansion of knowledge in this field over the past decade.
2. Increased survival among high-risk patients has resulted in a growing population with developmental delays and disorders and a greater need for societal resources over time.
3. Throughout childhood and adolescence, general cognitive ability is lower on average among individuals with congenital heart disease, even in those without a genetic syndrome. The magnitude of cognitive deficits increases with increasing disease severity.
4. Children with complex congenital heart disease experience an approximately 25% higher risk of substandard academic outcomes and are 50% more likely to require special educational services compared with children without congenital heart disease.
5. Deficits in attention and executive function skills are common in children with complex congenital heart disease, with a 4 times greater risk on standardized parent ratings and 2 times greater risk by teacher ratings.
6. Children 4-9 years of age with mild to severe forms of congenital heart disease have approximately 5-7 times higher odds for diagnosis or treatment for anxiety and depression compared with children without congenital heart disease.
7. The document proposes 3 categories of individuals with congenital heart disease at high risk of developmental delay or disorder. Risk Category 1 includes individuals undergoing cardiac surgery with cardiopulmonary bypass (CPB) during infancy. Risk Category 2 consists of individuals with chronic cyanosis. Categories 1 and 2 are both considered high risk. Referral for evaluation and early intervention is recommended.
8. Risk Category 3 includes individuals with increased neurodevelopmental risk who did not have infant CPB and were not chronically cyanotic. Patients without risk factors should have routine surveillance. Patients with risk factors should be referred for evaluation and early intervention. Risk factors include genetic abnormalities or syndromes, physiology associated with impaired cerebral blood flow, prematurity, postnatal diagnosis of congenital heart disease requiring surgery, perioperative seizures in infancy, significant brain injury on neuroimaging, need for cardiopulmonary resuscitation, extracorporeal membrane oxygenation for ventricular assist device, heart transplant socioeconomic disadvantage, significant parental psychological distress, feeding delay in infancy, growth failure in infancy or toddlerhood, and developmental delay in infancy or toddlerhood.
9. Proposed neuroprotective strategies include:
   • Prenatal diagnosis of congenital heart disease.
   • Optimal timing of delivery (full-term optimal).
   • Optimal timing of cardiac surgery (for example, later arterial switch procedures associated with increase neurodevelopmental risk).
   • Achievement of a good technical operation.
   • Minimizing deleterious effects of CPB.
   • Monitoring of cerebral perfusion and oxygen delivery.
   • Family-centered developmental care.
   • Supporting parents in their role as primary caregiver.
10. Evaluation of high-risk patients should be performed by qualified professionals who use an individualized and culturally sensitive approach.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Congenital Heart Disease

Keywords: Developmental Disabilities, Heart Defects, Congenital

< Back to Listings