What is Ebstein Anomaly?

Ebstein anomaly is a congenital heart disease seen in the tricuspid valve located between the right atrium and right ventricle in the heart. The tricuspid valve is not where it should be but lower down. Under normal conditions, the tricuspid valve has 3 leaflets. In Ebstein anomaly, the 2 leaflets of the tricuspid valve are displaced towards the right ventricle. While the other leaflet is usually located where it should be, sometimes it is adhered to the heart wall.

Due to Ebstein's anomaly, the tricuspid valve cannot function normally. Therefore, blood may leak back into the atrium due to the tricuspid valve not being able to open and close properly. Over time, the right atrium becomes larger than normal and the right ventricle becomes smaller than normal. The right atrium works harder to deliver blood to the right ventricle, causing the right ventricle to expand further. This enlarged part of the heart begins to weaken over time, causing heart failure.

The most common symptom of Ebstein anomaly in newborn babies and children is a bluish discoloration of the skin, called cyanosis. Cough, shortness of breath, slow growth and irregular heartbeat are among the symptoms that may be seen in childhood. Children with Ebstein anomaly may also have other heart defects, such as an atrial septal defect or pulmonary valve stenosis. Symptoms of Ebstein anomaly seen in adults include shortness of breath, fatigue, heart rhythm disturbances and chest pain.

In the treatment of Ebstein anomaly, the most appropriate treatment option is determined by taking other accompanying heart diseases into consideration. In some cases, direct surgical intervention is necessary.

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