Abstract
Objective. To study genetic and other factors affecting the results of operations for the repair of complete atrioventricular septal
defect in infants.
Material and methods. A total of 68 patients in the infant age underwent repair between 2009 and 2014. Of those, 58 (group I) had
Down syndrome (85.3%), 10 (group II; 14.7%) had normal chromosomes. In the analyzed groups in most cases age was about
3 months (р = 0.33). In both groups, the correction of the main congenital heart disease included two-patch technique.
Results. There were observed two deaths (only I group; 3.4%) in the early postoperative period but no difference in mortality was
found between groups (р = 0.52). It was found that the initial degree of mitral insufficiency component AV valve was more sever in
group 2 (р = 0.01). At discharge, the same parameter found even greater intergroup difference with the statistic value of the effect of
moderate degree (р =0.003; r =0.35). According to data of logistic regression analysis the statistically significant differences
between the groups were related to the substrate mitral insufficiency. The presence of Down syndrome was not found as a risk of
mitral valve reoperation because the actual freedom from mitral valve reoperation was 44% in group II compared to 100% in I group
(log-rank test p=0,03). Children without Down syndrome had significantly higher prevalence of reoperations as they had next factors:
not completely sutured cleft of neo-anterior mitral component (р = 0.001; OR, 40.8; 95% CI 6.9-269.5) and the lack of tissue
components of the bridging AV-leaflets (р = 0.001; OR, 27.4; 95% CI 4.8-143.1). Despite the lack of statistical difference in value of
the arterial pulmonary pressure (р = 0.3) according to the method of Kaplan-Meier in mid-term follow-up, in contrast, in group I there
are some patients have persisted pulmonary hypertension.
Conclusions. Although clinical outcomes were similar the long-term prognosis for the regression of pulmonary hypertension in children with Down syndrome is worse than children with a normal karyotype. However, the probability of re-operations on the mitral
component was higher in patients with a normal chromosome set due to the individual anatomical variations of the mitral valve in this
group of patients.
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