Cognitive, functional, educational achievement and behavioural measure
s were employed to assess neurobehavioral status in 57 of 60 participa
nts who were initially enrolled in the Baltimore-Washington Infant Stu
dy, and who survived surgical correction of complete transposition (co
ncordant atrioventricular and discordant ventriculo-arterial connectio
ns). Charts were reviewed to investigate the relationship between birt
h variables, surgical strategy and developmental outcomes. Higher preo
perative weight was associated with better outcomes on the Stanford-Bi
net Short-term Memory subtest, while lower preoperative oxygen tension
was associated with better outcomes on the Abstract/Visual Reasoning
subtest and a test of Visual-Motor Integration. Longer total bypass ti
me was associated with poor outcomes on the Short-term Memory subtests
. Higher average flow rates during cooling and rewarming were associat
ed with higher scores in the test of short term memory but poorer outc
omes on a test for visual motor integration. Longer cooling times were
associated with higher scores on the test for Visual-Motor Integratio
n. Patients suffering seizures scored lower on the Stanford-Binet Comp
osite, as well as in their tests of achievement. The data indicate tha
t non-verbal skills may be particularly sensitive to variations in sur
gical strategies employed to correct complete transposition. Overt neu
rological events, such as seizures, were related to global deficits in
intellectual functioning. Prospective studies evaluating systemic var
iations in surgical procedures and attempts to prevent and manage peri
operative neurological events are important for further investigation
of neurodevelopmental outcomes in children surviving surgical correcti
on.