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 Vol. 7 No. 7, July 2006
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NeoReviews Vol.7 No.7 2006 e363
© 2006 American Academy of Pediatrics

Neurodevelopmental Implications of Congenital Heart Disease

Michael A. Padula, MD, FAAP*
Anne M. Ades, MD, FAAP*

* Division of Neonatology, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, Pa

The first 300 words of the full text of this article appear below.


   Objectives
 
After completing this article, readers should be able to:

  1. Describe the causes of central nervous system injury for infants who have congenital heart disease.
  2. List genetic causes of congenital heart disease associated with developmental delay.
  3. Identify potential intraoperative mechanisms for neurologic injury for infants who have congenital heart disease.
  4. Identify risk factors for adverse neurodevelopmental outcomes for infants who have congenital heart disease.
  5. Describe which long-term developmental deficits are common among children who have congenital heart disease.


   Introduction
 
Among the more than 40,000 infants born with congenital heart disease (CHD) annually in the United States, one third require surgery during the first year after birth. (1) As advances in prenatal diagnosis, perioperative management, and surgical techniques have contributed to reduce rates of mortality, attention has shifted toward the neurodevelopmental morbidity among the growing cohort of survivors. Supportive surgical techniques, including cardiopulmonary bypass and deep hypothermic cardiac arrest (DHCA), have been a focus of outcome investigation, but mounting evidence suggests that multiple risk factors contribute significantly toward neurodevelopmental impairment. Long-term deficits in domains of cognition, attention, and neuromotor functioning among patients likely reflect the cumulative impact of features present before, during, and after surgical intervention.


   Preoperative Factors
 
     Genetic Implications
Genetic abnormalities and syndromes are present in many infants who have CHD and may be associated with neurodevelopmental delay (Table 1). Trisomy 21 affects 1 in 660 newborns; 40% of affected infants have CHD. Neurodevelopmental deficits, including hypotonia and mental retardation, are common among all patients who have trisomy 21. (5)(6) Among infants who have conotruncal defects, 18% have been found to have a microdeletion on chromosome 22. (17) Evaluation of children who have the 22q11 deletion, present in most infants who have velocardiofacial and DiGeorge syndromes, has revealed a unique profile of cognitive deficits. Impairments in visuospatial . . . [Full Text of this Article]






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Copyright © 2006 by the American Academy of Pediatrics.