Hereditary syndromes associated with the congenital heart diseases in Azerbaijan

• Main Author: N. A. Gadzhieva
• Format: Article
• Language: Russian
• Published: Ltd. “The National Academy of Pediatric Science and Innovation” 2018-03-01
• Series: Rossijskij Vestnik Perinatologii i Pediatrii
• Subjects: children; congenital heart disease; chromosomal pathological condition; down’s syndrome; monogenic syndromes; heterotoxic syndrome
• Online Access: https://www.ped-perinatology.ru/jour/article/view/623
• ISSN: 1027-4065; 2500-2228

This article is devoted to the study of the incidence and structure of the genetic syndromes associated with congenital heart diseases in Azerbaijan. The results of observation of 430 children with congenital heart diseases, which have been stayed in the Child Department of Scientific Surgery Center named after Academician M.A. Topchubashov during 2010-2015 period, have been analyzed. It was demonstrated that the incidence of the chromosomal and monogenic pathological conditions is 6.5±1.2% (28 children) among the above population. The chromosomal syndromes were diagnosed in 20 (4.7±1.0%) children, monogenic ones – in 8 (1.9+0.7%) children. The chromosomal pathological condition was mostly presented with the Down’s syndrome (in 12 patents). As to the monogenic syndromes, it was mostly the heterotoxic syndrome (4 children). These data testify that in spite of the multifactorial genesis of the most of the congenital heart diseases and role of the unfavorable factors of the antenatal period, the genetic component influences with a great importance upon the prevalence rate of the malformations.