Ebstein's anomaly

August 12, 2017 17:51 | Genetic Diseases

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 vice belongs Ebstein and refers to 1866

When this vice only one (front) wing of the tricuspid valve is attached properly, and the rest (back and septal) deformed, thickened, because of what can not function properly and create a formationvalvular insufficiency.The dimensions of the right ventricle decreased, it has a normal wall thickness.Pulmonary artery and its branches are not wide, due to decreased blood flow through them.Part of the right ventricle, together with an expanded as a result of an overload of blood volume of the right atrium forms a large cavity, the size of which is determined by the degree of tricuspid valve.Often there is a patent foramen ovale or atrial septal defect.The left side of the heart has not changed.

Circulatory disorders are determined by the degree of valve lesion, which leads to a reduced blood supply to t

he right ventricle and pulmonary artery.At the same time the blood of the right ventricle filling difficult, which is accompanied by an increase in pressure in the right atrium and the emergence of dumping excessive amounts of blood into the left atrium through a patent foramen ovale.A manifestation of this is the appearance of the blood discharge the child bluish coloration of the skin.

Ebstein's anomaly is diagnosed in the hospital or in the first weeks and months of life when expressed manifestations.A favorable variant of vice can remain asymptomatic for a long time, and it detected accidentally.Nearly 2/3 of children are cyanosis of the skin, often from birth, at least it appears between the 3rd and 12th years of life.Intensity cyanosis increases as the growing pressure in the right atrium and blood discharge to the left side of the heart.At the same skin color changes from crimson to dark blue.Some children do not have cyanosis.The dimensions correspond to the degree of cardiac circulatory disorders, increased mostly right, there is often a heart hump is located to the right of the sternum.When listening to the heart is determined by a characteristic noise, the intensity of which increases with inspiration.Heart failure arises sometimes from the very first months of life, but may first appear very late.A characteristic feature of Ebstein's anomaly with significantly enlarged right atrium are arrhythmias, which is manifested by sudden onset seizures palpitations.

additional methods of examination of children are ECG, PCG (graphic check heart sounds), chest X-ray, ultrasound and heart.The diagnosis is confirmed during catheterization of heart cavities and the introduction of the right ventricle contrast medium.

prognosis of Ebstein's anomaly is defined by blood circulation disorders.Most patients die at the age of 20-40 years.In terms of the forecast are unfavorable complications such as increased size of the heart (cardiomegaly), heart failure, heart rhythm disorders.

children with asymptomatic defect surgery is not carried out.In severe cases, make prosthetic tricuspid valve plasty and atrial septal defect.