Синдром Ятагана: Подходы К Диагностике И Ведению

Abstract

Âèïàäîê ³ç ïðàêòèêè / Case ReportDespite the continuous improvement of the methods of prenatal diagnosis of fetal malformations, the limited possibilities of intrauterine verification of vascular anoma lies of the lungs still take place. One of the most rare and difficult to diagnose forms of combination of vascular anomalies is partial abnormal pulmonary vein drainage, which is sometimes a component of scimitar syndrome (ICD-10: Q26.8) [1].Scimitar syndrome (hypogenetic pulmonary syndrome, venolobar syndrome or "yataghan" syndrome) belongs to a group of venolobar syndromes associated with lung sequestration and congenital heart disease. This is a congenital anomaly characterized by the fact that one or more pul-monary veins drain not into the left atrium, as normal, but directly into the inferior vena cava above or below the diaphragm. Scimitar syndrome accounts 4 % of abnormal pulmonary vein drainage. This syndrome is associated with changes in the arterial supply of the lungs, the right lung is hypoplasic, as well as the hypoplasic right bronchus and the right pulmonary artery. As far as the heart is concerned, in addition to dextroposition of the heart with displacement of the mediastinum, the most frequent changes (1/4 cases) are the absence of the pericardium, and other defects of development, such as defect of the atrial (60-70 %) or interventricular septum, coarctation of the aorta and tetralogy of Fallot (25 %).