[Coexistence of congenital heart diseases with other congenital defects].

Aim: Evaluating the prevalence and type of non-heart malformations in patients hospitalised in the Department of Pediatric Cardiology of the Children's Memorial Health Institute due to congenital pathology of the circulatory system; comparing the frequency of other congenital malformation in the group researched in relation to the population overall and evaluating their relevance to survival.

Material And Methods: Research included 266 infants with heart defects. The patients' data were analysed in retrospect on the basis of medical records, taking into account the kind of heart defect, defects of other systems, and mortality in the first year of life. The frequency of non-heart congenital defects were compared with data from the Polish Registry of Congenital Malformations.

Results: In 71 children among 266 patients congenital defects of other systems were diagnosed. The group researched presented a significantly greater occurrence of such defects than the population overall (26.7% vs 1.81%). The most frequent diagnoses were: 1. chromosomal abnormalities (N=26; 9.77%), 2. malformations of the digestive system (N=7; 3.01%), 3. syndromes of multiple congenital defects (N=6 ; 2.26%). The defects usually coexisted with the atrio-ventricular septal defect (18 out of 22 patients - 81.8%) and rarely in neonates with duct-dependent defects (19 out of 122 patients - 15.6%). 32 children died, of whom 10 had congenital non-heart malformation in addition to congenital heart defects. No significant difference in the frequency of death was found in children who presented only with heart pathology in comparison to those with other defects (11.2% vs 14%).

Conclusions: In patients with congenital heart defects other congenital malformations occurred more often than in all the population, especially chromosomal abnormalities, malformations of the digestive system and congenital malformation syndromes. Congenital malformations were not connected with higher mortality of children with heart defects.