Repair of complete atrioventricular septal defect in infants with down syndrome: outcomes and long-term results.

Abstract

In clinical practice, the combination of congenital heart disease (CHD) with malformations of other organs occurs in about 10 % of cases, including chromosomal disease with heart defects, which are observed mainly with certain syndromes. In the Bakoulev SCCS (Moscow, Russian Federation), from 01.2005 to 01.2011, complete atrioventricular septal defect (CAVSD) repair was performed on 163 patients (5.6 ± 3.0 months) with Down Syndrome (DS) using the single-patch (n = 40) and the two-patch (n = 123) methods. The control group consisted of 214 infants aged 6.49 ± 3.03 months with CAVSD and normal karyotype. A retrospective cohort study was made, as well as a comparative analysis of the immediate (up to 30 days) and long-term (12-75 months, at the average of 56 ± 15) results of the repair of CAVSD in infants with DSand normal karyotype/chromosome set (NK). During the hospital treatment period, we registered the following complications: pulmonary hypertensive crises in 6 % (n = 9) of patients with DS and in 10 % (n = 21) of infants with NK, infectious complications in 21% (n = 34) of patients with DS and in 8% (n = 17) of infants with NK. Squeal structures in groups were differentiated. The doses and duration of cardiotonic support in the NK patients were significantly higher in comparison with the DS patients (7.5 ± 2.1 days vs 3.4 ± 1.15 days, p < 0.05). Respiratory infections on the background of immunodeficiency were found more often in the DS group (21% in DS vs 8% in NK, p < 0.05), demanding higher postoperative pulmonary ventilation time in DS patients in comparison with normal infants was required (DS 5.1 ± 2.8 days vs NK 1.7 ± 0.8 days, p < 0.05). In DS infants, abnormalities of the left AV valve (doubling of the mitral valve, single papillary muscle, closely spaced groups of papillary muscles, leaflet or chordal dysplasia, hypoplastic valve ring) occur as statistically significant (8% DS vs 12% NK; p < 0.05) which is rarer than in children having the same defect, but without Down syndrome. Concerning the long-term results, there was no significant difference (Gehan-Wilcoxon test) in actuarial freedom from reoperation after repair of CAVSD between DS and NK groups (p < 0.13). However, the presence of Down Syndrome in patients significantly increases the risk of severe co-morbidities that have a significant impact on the recovery period, as well as on life expectancy even after successful CHD correction.