RARE CONGENITAL DISEASES IN CHILDREN'S CARDIAC SURGERY

Aim. To conduct an analysis of the own material on a study of rare diseases in pediatric cardiology and cardiac surgery. Material and methods. The study included 18 newborns and children observed for the period of 2010-2019. All children underwent standard examination methods: ultrasound examinations of the heart and blood vessels (Echocardiography); angiocardiography; ECG; CT according to indications; laboratory and histological studies; macro preparations examination; “dry drop” method for congenital metabolic diseases. Results. Examined patients included: 5 newborns; 5 patients up to 1 year; 8 patients up to 5 years. Following rarely encountered diseases were found: multiple heart rhabdomyomas - in 6 patients (33%), one of whom had confirmed congenital tuberous sclerosis (Pringle-Bournville disease); an isolated giant cyst of the right atrium-1 (5.5%), mitral and aortic valve defects with the development of marked myxematous deformation and functional insufficiency were found in 6 children with mucopolysaccharidosis (33.3%); mitral valve fibroma in a newborn -1 (5.5%); isolated right ventricular hypoplasia - 1 case (5.5%); 1 case with the Marfan triad (5.5%). The features of clinical and instrumental examination of these patients categories are presented. Conclusions. Based on the results of the analysis, we consider the necessity of a multidisciplinary approach for children with complex congenital defects of heart and blood vessels, especially with comorbidities. This is necessary to consider choosing the tactics and timing of operations on the heart and blood vessels, dynamic observation and the medication prescription.

congenital heart defects, right ventricular hypoplasia, cardiac surgery, cardiology, mucopolysaccharidosis, newborns, rhabdomyoma, tuberous sclerosis

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