TOTAL ANOMALOUS VENOUS CONNECTION
Introduction. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac defect that occurs in less than 1% of people with congenital heart disease. It is a life-threatening defect, and without surgical treatment, few individuals survive past the first year of life. There are no known fetal environmental variables associated with TAPVC. One case series reported a 3:1 male preponderance for TAPVC to the portal vein, but not for other TAPVC locations.
Aim. In this scientific review, a comparative analysis of literature data on the epidemiology, etiology, pathogenesis of the disease, the main methods of treatment for various types of total anomalous pulmonary vein drainage was carried out.
Search strategy: Literature search was carried out in the electronic databases PubMed, MEDLINE, Web of Science, Google Scholar and e-library, using keywords (congenital heart disease, total anomalous venous connection, anatomy, morphology, embryology). Relevant data reflecting the features of this defect were taken for description in the review. Of all the selected articles for further analysis, 26 sources were included that met the inclusion criteria and excluded duplication or repetition of information.
Results. Several crucial variables have a significant impact on the etiology and clinical presentation of TAPVC. The most crucial component is the presence or absence of blockage at any level of the vein system. In blocked forms, elevated pulmonary venous pressure causes a rise in hydrostatic pressure in the capillaries, resulting in pulmonary edema. A surgical treatment is accepted as urgent if a patient also has an obstruction. Imminent fatality after surgical reconstruction is from 2% to 20%. A success between 80% and 98% can be reached after surgical treatment due to the analysis of the case series and the presence of venous obstruction
Conclusion.Knowledge of the causes of development and pathogenesis, clinic and surgical methods of treating TAPVC will allow us to detect this pathology at an early stage, to understand and improve the tactics of managing the patient, and to choose the tactics of surgical treatment correctly.
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Bastarbekova L.A., Rakhimzhanova R.I., Dautov T.B., Bimakhan A.Zh., Kaliyev B.B., Moldakhanova Zh.A. Total anomalous venous connection // Nauka i Zdravookhranenie [Science & Healthcare]. 2023, (Vol.25) 2, pp. 175-182. doi 10.34689/SH.2023.25.2.012Related publications:
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