Structural features of the heart in children with arrhythmic syndrome due to nonspecific connective tissue disorder

Introduction. Connective tissue is highly organized system, its disorders are characterized by a pronounced polymorphism of morphological and clinical manifestations. The cardiovascular pathology in patients with nonspecific connective tissue disorder attracts attention due to the high risk of complications: rhythm and conduction disorders, infective endocarditis, vascular thromboembolism and sudden cardiac death. Therefore, it’s very important to use up-to-date equipment and methods of early diagnosis of a high risk of fatal events in young. Aim. To investigate the structural features of the heart and their relationship with the process of myocardial remodeling in children with arrhythmic syndrome and nonspecific connective tissue disorder. Materials and methods. Sixty-five children were examined, 40 of them had arrhythmic syndrome in combination with nonspecific connective tissue disorder, and 25 had only minimal manifestations of nonspecific connective tissue disorder without arrhythmic syndrome. Such up-to-date methods as assessment of myocardial remodeling and longitudinal strain, natriuretic peptide assessment were included in the diagnostic algorithm. Results and discussion. The study showed a variety of phenotypic and visceral markers of nonspecific connective tissue disorder in children with arrhythmic syndrome. Arrhythmic syndrome was manifested by monotopic and heterotopic heart rhythm disorders, and structural changes of the heart: mitral valve prolapse and myocardial wall thinning were correlated with a more frequent increase in natriuretic peptide. Speckle-tracking echocardiography showed a significant decrease in longitudinal myocardial strain with predominance of myocardial strain in the anterior basal segment in children with arrhythmic syndrome. Conclusion. Our study showed a variety of phenotypic and visceral markers of undifferentiated connective tissue dysplasia in children with arrhythmic syndrome. The data obtained require further mathematical analysis and the establishment of a possible relationship between the external manifestations of the disease and cardiac rhythm and conduction disturbances.