Аномалия Эбштейна

Abstract

Ebstein’s anomaly is a rare congenital malformation characterized by apical displacement of the right atrioventricular ring with an anomaly of the development of the tricuspid valve and the frequent presence of other heart defects. According to our data, this defect was diagnosed in 2 cases in more than 6 000 sectional studies: in an adult and a child. According to the echocardiography rooms of non-specialized multidisciplinary children's hospitals and children's polyclinics, the defect is detected with a frequency of 1:24.000–1:32.000 studies. Genetic and environmental factors (viral infections, benzodiazepines, lithium) are indicated as probable causes. Clinical signs are cyanosis, shortness of breath, heart failure, cardiac arrhythmias. Morphologically there are abnormalities of the tricuspid valve, papillary muscles and chordal apparatus, right ventricular myocardial hypertrophy, thinning of the walls of the right atrium and atrialized part of the right ventricle. Decisive for the diagnosis is ultrasound. Displacement of the right atrioventricular ring is revealed. The degree of bias determines the severity of the defect. The amplitude of the tricuspid valve movement is increased. Its valve closure occurs later than the mitral. A 40 ms difference between the tricuspid and mitral valve closure points is a diagnostically important sign of Ebstein’s anomaly. The tactics of a doctor are determined by the clinical picture