Ebstein's anomaly

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Ebstein's anomaly

Ebstein's anomaly (pronounced: /ˈɛpstaɪn/), is a rare congenital heart defect characterized by the malformation of the tricuspid valve and the right ventricle. The condition is named after Wilhelm Ebstein, a German physician who first described the anomaly in 1866.

Etymology

The term "Ebstein's anomaly" is derived from the name of its discoverer, Dr. Wilhelm Ebstein. The word "anomaly" comes from the Greek word 'anomalía' meaning 'irregularity' or 'deviation from the norm'.

Definition

Ebstein's anomaly is a congenital heart defect where the tricuspid valve is displaced towards the apex of the right ventricle. This displacement results in the formation of an 'atrialized' portion of the right ventricle, which is functionally part of the right atrium but morphologically part of the ventricle.

Symptoms

Symptoms of Ebstein's anomaly can vary greatly, depending on the severity of the defect. They may include shortness of breath, cyanosis (bluish discoloration of the skin due to low oxygen levels), heart palpitations, and fatigue. In severe cases, it can lead to heart failure or arrhythmia.

Diagnosis

Diagnosis of Ebstein's anomaly is typically made through echocardiography, a type of ultrasound used to create images of the heart. Other diagnostic tests may include electrocardiogram (ECG), cardiac MRI, and cardiac catheterization.

Treatment

Treatment for Ebstein's anomaly depends on the severity of the condition. It may include medications to manage symptoms, cardiac surgery to repair the valve, or in severe cases, heart transplantation.

Related Terms

External links

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