CLINICAL AND EPIDEMIOLOGICAL SIGNIFICANCE SYNDROME OF CONNECTIVE TISSUE DYSPLASIA IN THE FORMATION OF CONGENITAL HEART DEFECTS. REVIEW
Introduction. Structure of all congenital malformations include 30% malformations of the heart and blood vessels (CHD - congenital heart disease), congenital malformations make a significant contribution for child mortality and disability, requiring significant economic costs for surgical correction and social assistance for children with disabilities. In the Republic of Kazakhstan, practical measurement of CHD among live births is 8-10 per 1,000 live births and every year the total number of live births children with CHD in Kazakhstan is 3,000. The aim of study was analyzed of literature data of congenital heart defects and studied the problem of the CHD associated with connective tissue dysplasia. Search strategy. Search of literary sources was conducted in the bases of PubMed, Google Academy, Tompson Reuters. A total of 70 sources were studied by keywords (congenital heart disease, connective tissue dysplasia, prevalence of congenital heart defects, minor cardiac abnormalities, connective tissue). The criteria for inclusion in the review were statistical, clinical data on the prevalence of pathology in the World and in the Republic of Kazakhstan. Exclusion criteria are congenital heart defects due to gene mutations. Results. The review presents: the mechanisms of dysplasia of connective tissue at the present stage, connective tissue dysplasia of the heart, the prevalence of congenital heart diseases in children in the World, and in the Republic of Kazakhstan. Conclusion of the research work show that the anomaly of collagen developed of connective tissue dysplasia, which at the present anomaly of collagen occupies one of the leading places in the structure of cardiovascular diseases of childhood. At the same time, the contribution the anomaly of collagen to the assessment of the severity of the CHD is not sufficiently studied. The research of Kazakh and foreign scientists testify about uniqueness of the structure and function of the connective tissue in organism. Influence of exogenous and endogenous factors, conditions for the onset of congenital heart defects are created during ontogeny. The study of the mechanisms of such destructions is relevant at the present time.