This article presents a clinical case of the follow-up of a patient with a congenital heart defect in the form of the pulmonary artery stenosis in the setting of the "sluggish skin" syndrome. It is shown that the occurrence of congenital defects largely depends on the hereditary factor, which, in this case, is a related marriage. You should also pay attention to the fact that the associated congenital heart disease: the mouth of the pulmonary artery against the background of the "sluggish skin" syndrome spontaneously stopped without surgery, which is probably due to the peculiarity of the background disease, that is, generalized elastolysis. The natural course of this congenital heart defect against the background of a genetic pathology in the form of "sluggish skin" syndrome indicates the positive influence of the pathological substrate (connective tissue hyperelasticity) on the state of the tissues of the heart and blood vessels. Despite the already available data, it is still difficult to make a prediction of the course of this disease. For a better understanding of the course of this pathological process, further observation of the patient is necessary.
Fallo's tetrad -one of the most often found congenital heart disease. The relevance of a problem of complications after cardiac operations at the Fallo's tetrad remains acute for today. The analysis of results of expeditious treatment and complications will allow to solve problems of timely diagnostics, treatment and prevention of the reasons of an unsatisfactory clinical condition of patients at all stages of postoperative maintaining.Objective: Studying the structure of postoperative complications, determination of the causes of postoperative complications at a hospital stage at patients after a definitive and palliative repair of Fallo's tetrad definition of diagnostic and medical tactics in the postoperative period.Material and methods: The studied patients with Fallo's tetrad (126 children aged from 2 days till 9 years, from them boys -64, girls -62) were distributed on 2 main groups: after the definitive repair of defect (DRD) and after the palliative correction of defect (PCD). The complex assessment of postoperative complications at patients with TF was carried out with use of clinical-laboratory research methods.Results: Complications at a hospital stage arose in 38 (45.2%) cases from 84 (100%) patients after a definitive repair and in 17 (50%) cases from 34 (100%) -after palliative correction of Fallo's tetrad. The structure of complications after a definitive and palliative repair of defect is defined. The reasons and risk factors of development of postoperative complications after surgical correction of Fallo's tetrad are defined. Features of a clinical current of complications after a definitive and palliative repair of Fallo's tetrad are noted. Optimum tactics in treatment of postoperative complications at a hospital stage after a definitive and palliative repair of Fallo's tetrad is defined.
Article provides review of the available world literature on performing surgical correction of the atrioventricular septal defect in patients with Eisenmenger syndrome such as: basic pathophysiological principles, technique, indications, clinical and hemodynamic results. Currently, according to the literature, there is no clarity in the choice of a method for correcting severe congenital heart defects in combination with Eisenmenger syndrome (one -or two-step approach). The article presents the results of studies of both one-and two-stage approaches to correction of the atrioventricular septal defect in this category of patients with their advantages and disadvantages.
In the century of advanced technology, medicine has leaped forward, which contributed to the possibility of correcting such severe congenital heart defects in children as the transposition of great vessels. However, despite the progress in medicine and compliance with the rules of asepsis and antisepsis, infection of the postoperative wound remains one of the frequent and terrible postoperative complications, worsening the prognosis and increasing the risk of mortality. In this retrospective analysis, we consider a clinical case of a radical correction of the great vessels transposition with a complication that has arisen (wound infection) in the early and late postoperative period. The relevance of the article lies in the fact that we studied international experience in managing patients with this complication and applied it in this clinical case in a child with transposition of great vessels, who underwent several cardiac and thoracic operations complicated by wound infection, sepsis, DIC-syndrome with significant deficit bone skeleton of the chest. The child's condition improved significantly: infectious complications were stopped, signs of heart failure decreased. The child is observed for 7 years, the observation will continue.
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