In case of hypermobility joints syndrome, a high incidence of valvular and arrhythmic manifestations of heart connective tissue dysplasia is shown, their features and spectrum are studied. Aim of the research. To estimate the prevalence and spectrum of valvular manifestations of cardiac connective tissue dysplasia in hypermobility joints syndrome patients and their possible association with the features of the orthopedic status. Material and methods. 214 patients with hypermobility joints syndrome were examined in comparison with the control group (n = 40). X-ray and X-ray investigations of the spine, thorax, MRI of the spine, and joints were performed according to indications. For the verification of dysplastic valve syndromes, heart rhythm disturbances EchoCG, resting ECG and 24-hour ECG monitoring were used. Results. Polyfocal and combined skeletal dysplasias in hypermobility patients’ phenotype are worked out in details. The prevalence and nosological characteristics of thorax and spine deformations, increasing the risk of unfavorable course of cardiac dysplastic syndromes with the formation of structural hemodynamic disorders and complications, have been fixed. Conclusion. The presence in the phenotype of hypermobility persons of polyfocal and associated skeletal dysplasia, predisposing to clinically significant deformations with a possible cardiac and large vascular trunks disposition classifies them as a risk group for the development and adverse course of cardiac dysplastic syndromes.

Keywords: joint hypermobility syndrome, connective tissue dysplasia, valvular syndrome, arrhythmias