Ebstein anomaly

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Ebstein anomaly (pronounced: EHP-steen uh-NOM-uh-lee) is a rare heart defect that is present at birth (congenital). It primarily affects the tricuspid valve, one of the heart's four valves, which prevents blood from flowing back into the right upper heart chamber (atrium) from the right lower heart chamber (ventricle).

Etymology

The condition is named after Wilhelm Ebstein, a German physician who first described the condition in 1866.

Definition

Ebstein anomaly is characterized by a malformed tricuspid valve that sits lower than normal in the right ventricle. This results in a portion of the right ventricle becoming part of the right atrium (atrialization of the right ventricle), leading to a variety of complications.

Symptoms

Symptoms of Ebstein anomaly can vary greatly from person to person. They may include shortness of breath, especially with exertion; fatigue; a heart murmur; cyanosis (a bluish discoloration of the skin caused by low oxygen levels); heart palpitations; and in severe cases, heart failure.

Diagnosis

Ebstein anomaly is typically diagnosed through a combination of physical examination, medical history, and imaging tests such as an echocardiogram, MRI, or cardiac catheterization.

Treatment

Treatment for Ebstein anomaly depends on the severity of the condition and the presence of symptoms. It may include medications to manage symptoms, procedures to correct the heart rhythm, and in severe cases, heart surgery to repair or replace the tricuspid valve.

Prognosis

The prognosis for individuals with Ebstein anomaly varies widely. Some people may live with few symptoms and require minimal treatment, while others may need multiple surgeries and have a reduced life expectancy.

See also

External links

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