CLINICAL AND EPIDEMIOLOGICAL SIGNIFICANCESYNDROME OF CONNECTIVE TISSUE DYSPLASIAIN THE FORMATION OF CONGENITAL HEART DEFECTS.REVIEW.

Abstract

Введение. 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 patholog y
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.