Outcomes of Congenital Heart Disease: A Review

Angela Green


Pediatr Nurs. 2004;30(4) 

In This Article

Neurodevelopmental and Cognitive Outcomes

As a group, children with CHD have higher rates of neurodevelopmental abnormalities than their peers. The sources of these abnormalities are multiple. Structural brain abnormalities and microcephaly are more common in children with CHD, as are genetic syndromes associated with developmental delays. Infants with complex CHD may experience preoperative neurologic injury as a result of intraventricular hemorrhage or decreased cerebral perfusion. The use of cardiopulmonary bypass and/or deep hypothermic circulatory arrest intraoperatively increases the risk of neurodevelopmental abnormalities (Mahle, 2001).

Canadian researchers have reported preoperative neurobehavioral abnormalities in more than half of newborns and in 38% of infants with CHD. Abnormalities persisted in most of these infants in the immediate postoperative period (Limperopoulos et al., 2000). Among children less than 2 years of age who were followed 12-18 months post cardiac surgery, abnormal neurologic examinations were reported in 41%. Abnormalities included gross and/or fine motor delays (42%) and global developmental delays (23%). Risk factors for deficits included increased length of stay in the intensive care unit, microcephaly, deep hypothermic cardiac arrest, acyanotic lesion, palliative (as opposed to corrective) surgery, abnormal perioperative neurobehavioral status, increased number of subsequent admissions, and weight below the second percentile (Limperopoulos et al., 2002). One U.S. study reported that more than 46% of children with CHD requiring surgical or catheter intervention received abnormal ratings on developmental screening. These children were significantly more likely to be rated as abnormal than children with hemodynamically insignificant CHD. Again, specific delays included gross and fine motor developmental delays. Personal and social skill delays were also apparent in this group (Weinberg, Kern, Weiss, & Ross, 2001). Most studies have excluded children with known syndromes or central nervous system abnormalities; thus, the incidence of abnormalities in this population is probably higher.

Forbess and colleagues (2002) reported that mean intelligence quotient (IQ) scores of children with CHD were within 1 standard deviation of the normal range. Mean scores on a memory and learning screening tool were also within normal range. The percentage of children with abnormal scores was not reported; however, risk factors for low full scale IQ included velocardiofacial syndrome, lower socioeconomic status, single ventricle anatomy, longer postoperative intensive care unit stay, and cumulative duration of hypothermic circulatory arrest. Of note, children with other defects affecting cognition were excluded (Forbess et al., 2002). In a study of adults with CHD, the mean IQ also was normal. However, 12.7% of the sample were mentally retarded or had borderline intellectual functioning, and cyanotic heart disease was a risk factor for lower IQ scores (Utens, Bieman, et al., 1998).


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