The ebstein anomaly
Ebstein's anomaly is a malformation of the tricuspid valve. The frequency of its occurrence is 0.5-0.7% of all congenital heart defects. The first description of this defect belongs to Ebstein and refers to 1866.
In this defect, only one( front) leaf of the tricuspid valve is attached normally, and the remaining( posterior and septal) are deformed, thickened, which can not normally function and form a valveinsufficiency. Dimensions of the right ventricle are reduced, its wall has a normal thickness. The pulmonary artery and its branches are not very wide, which is explained by the reduced blood flow through them. Part of the right ventricle together with the right atrium enlarged as a result of an overload with blood volume forms a large cavity, the size of which is determined by the degree of insufficiency of the tricuspid valve. Often there is an open oval window or defect of the interatrial septum. The left part of the heart is not changed.
Circulatory disturbances are determined by the degree of damage to the valve, which leads to a decreased blood flow to the right ventricle and pulmonary artery. At the same time, the filling of the right ventricle with blood is difficult, which is accompanied by increased pressure in the right atrium and the emergence of the discharge of excess blood into the left atrium through an open oval window. The manifestation of such a discharge of blood is the appearance in the child of cyanotic staining of the skin.
Ebstein's anomaly is diagnosed in the maternity home or in the first weeks and months of life with pronounced manifestations. A favorable variant of the defect can remain asymptomatic for a long time, and it is detected accidentally. Almost two thirds of children have cyanotic skin, often from birth, less often it appears between the 3rd and 12th years of life. The severity of cyanosis increases as pressure increases in the right atrium and discharge of blood into the left heart. Thus the shade of a skin varies from crimson to dark blue. Some children have cyanosis. The size of the heart corresponds to the degree of circulatory disturbance, increased mainly to the right, often there is a heart hump, located to the right of the sternum. When listening to the heart, a characteristic noise is determined, the intensity of which increases with inspiration. Heart failure occurs sometimes from the first months of life, but it may first appear very late. A characteristic feature of the abnormality of Ebstein with significantly increased right atrium is the disturbance of the rhythm, which is manifested by the sudden occurrence of attacks of rapid heartbeat.
Additional methods of examining children are ECG, PCG( graphic recording of cardiac murmurs), radiography of the chest, UZ and heart. The diagnosis is confirmed by cardiac catheterization and the introduction of a contrast medium into the right ventricle.
The prognosis for Ebstein's abnormality is determined by impaired blood circulation. In most cases, patients die at the age of 20-40 years. In terms of the prognosis, such complications as cardiac enlargement( cardiomegaly), heart failure, heart rhythm disturbances are unfavorable.
Children with asymptomatic defects are not treated surgically. In severe cases, a tricuspid valve prosthesis and an atrial septal defect are made.