Objective: The purpose of this study was to evaluate the results of treatment in patients with deformities of the spine and congenital heart defects. Methods: Eighty-seven children aged 10 to 18 years old (27 males and 60 females) were treated surgically due to scoliosis. Results: The minimum angle of the curvature arc was 68°. The deformity parameters were evaluated on the basis of the spine x-ray. In patients with combined and rigid forms of scoliosis, correction was performed using dorsal segmental instrumentation. Where there was rigid deformity and it was not possible to perform a front release according to the somatic indications, a one-stage dorsal correction was performed in conditions of halo traction in combination with SPO or Ponte osteotomy. In patients with moderate impairment of blood circulation in the presence of rigid curvature of the main arch, two-stage surgery was performed, with ventral release at the apex of deformation, followed by halo traction. As a second stage, dorsal correction and stabilization of the spine was performed. In the operated patients, the mean correction with dorsal instrumentation was 64.2%. An average of 12 vertebrae were included in fusion. The degree of apical vertebral body derotation was found to be up to 30% of the initial rotation. With the use of hybrid and hook systems, the average degree of derotation was 18%. According to the physical examination within one year, the parameters of the function of external respiration and bronchial permeability increased significantly, from 10% to 30%, which indicates the functional nature of changes in cardiorespiratory disorders. Level of evidence IV, Case series.