These changes in the deformations may be the first signs of deterioration of the left ventricular function and the existence of primary cardiomyopathy in young adults with mitral valve prolapse, which may be caused by increased transforming growth factor-β signalling.

To study the possibility of using thrombin generation tests in platelet-rich and platelet-poor plasma for evaluation of dual antiplatelet therapy efficacy in patients with coronary artery disease (CAD), following percutaneous coronary intervention. Venous blood was analyzed from CAD patients aged 53-75 years who had undergone percutaneous coronary intervention with stenting within one year and had been receiving standard doses of clopidogrel and aspirin (75 and 75-100 mg per day, respectively). The control group comprised age- and sex-matched subjects without clinical signs of CAD who were not receiving these drugs. Thrombin generation tests were performed in platelet-rich and platelet-poor plasma. Intravascular platelet activation, induced platelet aggregation, and routine coagulation were evaluated. Antiplatelet treatment did not influence results of routine coagulation tests or intravascular platelet activation. The dual antiplatelet therapy affects collagen-induced platelet aggregation (44 ± 2.5 vs. 7.9 ± 2.6%, р = 10) and leads to decreases in endogenous thrombin potential (1900 ± 85 vs. 1740 ± 95 nM∙min, p = 0.0045), maximum thrombin concentration (134 ± 9.5 vs. 106 ± 6.5 nM, p = 4∙10), and increases in time to peak thrombin (27 ± 1.5 vs. 31 ± 2 min, p = 0.0012). Decreases in thrombin generation rate showed the highest statistical significance (13 ± 2 vs. 7.9 ± 0.8 nM/min, p = 10). Antiplatelet treatment did not alter thrombogram parameters for platelet-poor plasma.

The article describes the current algorithms of diagnostics of inherited (dysplasia) of connective tissue (DCD) that are currently in force in the territory of the Russian Federation, compared in terms of identification of morpho-functional cha racteristics of heart. Describes the shortcomings of these algorithms do not take into account the gender features of the prevalence of signs. According to the algorithm, developed by experts of Russian Society of Cardiology (RSC), highlight dysplastic phenotypes, most of which is now a marfanoid habitus (MH). Revision of the diagnostic algorithm, which takes into account the specificity of individual MH bone signs and their gender dimensions. It is proved that for young people with MH is characterized by increased frequency of cardiac anomalias (atrial Septal aneurysm, the functional asymmetry of the aortic valve), identified structural features heart-relative myocardial hypertrophy of the left ventricle, increasing the diameter of the aorta. The features of heart rate in patients with MH – relative tachycardia during daylight hours, reducing the symptoms of sinus arrhythmia, decreased heart rate variability power spectral. It is shown that for boys with MH is characterized by lengthening the electrical systole (QT interval), 21.4% of cases greater than 480 Ms. At the same time, the DCD diagnostic algorithm that is described in the recommendations of the Russian Scientific Society of therapists (RSSIM leads to over diagnosis this condition in young age does not allow to identify the structural and functional features of the heart and could not be recommended for practical use.

No abstract

According to contemporary views, hereditary connective tissue disorders divided classified Marfan syndrome, Loeys-Dietz’s, Ehlers-Danlos syndrome, the primary mitral valve prolapse. It is known that the fibrillinopaty, which include the Marfan syndrome and Loeys-Dietz’s is characterized by activation of TGF-β signaling pathway. With high le vels of TGF-β attributed most of these clinical manifestations these diseases – aneurysm of the aorta, arahnodaktylya, duralectasy. Assessment of the activity of TGF-β in persons with marfanoid habitus has not previously been studied. Materials and methods. As part of this work, surveyed 70 people: 61 patients young age (median age of 20.1 ± 2.1 years), among which 36 boys and 25 girls and 9 men with verified diagnosis Marfan syndrome (median age 27.9 ± 9.3 years). All survey performed Echocardiography with a targeted search of small anomalies of heart. Results. Correlation analysis showed a direct and reliable connection between arahnodaktylya and concentration of TGF-β1 in serum (r = 0.4, p = 0.05). For young people with signs of marfanoid habitus are characterized by reliably a higher concentration in the serum of both isoforms of TGF-β. Excess of threshold levels of TGF-β1 revealed at 20% of the core group and not found at all in the control (p < 0.05). Among persons with exceedances of threshold values for at least one faction of the TGF-β patients with signs of marfanoid habitus met almost three times more often than in the group with normal values of TGF-β (p = 0.01, χ2 = 5.58). In the group of persons with marfanoid habitus and increases TGF-β are detected more frequently such as atrial septal aneurysm, false chord left ventricle papillary muscles, incremental, deflection of shutters of the mitral valve in 1-2 mm, asymmetry tricuspid aortic valve.