ДИСПЛАЗИЯ СОЕДИНИТЕЛЬНОЙ ТКАНИ КАК КОНСТИТУЦИОНАЛЬНАЯ ОСНОВА ВРОЖДЕННЫХ ПОРОКОВ СЕРДЦА У ЖЕНЩИН И ИХ НОВОРОЖДЕННЫХ ДЕТЕЙ

The relationship between the cardiac pathology associated with connective tissue dysplasia in children and that of their parents require new approaches to their diagnosis and prevention. The purpose of research is the improving preclinical diagnosis of complications of pregnancy in women with the congenital heart disease and connective tissue dysplasia based on a comprehensive evaluation of the clinical and metabolic parameters and development of measures for the prevention of desadaptation of newborn children. Materials and methods. According to the changes in the major markers of collagen of the extracellular matrix of connective tissue: a free, peptidnosvyazanny and beloksvyazanny hydroxyproline in the serum of 50 pregnant women, patients with congenital heart disease with the presence of phenotypic signs of connective tissue dysplasia, and without them, during the gestational period can monitor the metabolism of this protein. Results of the study. The gestational age of 16 weeks the concentration fractions of hydroxyproline in pregnant women with congenital heart disease is different from the concentration of healthy pregnant women, with fluctuations from 1,62 to 5,6 mg/l. At 28–30 weeks of gestation in female patients in the presence of connective tissue dysplasia a level of peptidnosvyazanny hydroxyproline increases by 1,2 times, which is characterized by initial signs of disturbances in the mother-placenta-fetus, but in 35–40 weeks the concentration of free hydroxyproline increases, ratio free / peptidnosvyazanny hydroxyproline reduces, which may be a poor prognostic sign for the fetus and newborn. A similar trend is observed changes in collagen markers in newborns diagnosed with the congenital atrial septal defect, which also the indicates slowing metabolism of collagen and reduction in the speed of biological recycling of this protein. Conclusions. The biochemical markers may be used as additional criteria for preclinical diagnosis of cardiac pathology of the fetus and newborn, assessment and prediction of their current prevention measures, and dysplasia of the connective tissue can be seen as the constitutional basis for congenital heart disease in women and their newborn children.