Ebstein Anomaly

Ebstein Anomaly is a rare heart defect that affects the tricuspid valve, which controls blood flow between the heart's right atrium and right ventricle. In this condition, the valve is abnormally formed and positioned lower than usual, causing blood to leak backward. This can lead to an enlarged right atrium and reduced heart efficiency. The main health impact is difficulty pumping blood properly, which may cause symptoms like heart murmurs, fatigue, and cyanosis (bluish skin). It primarily affects the cardiovascular system and can vary widely in severity from mild to life-threatening. Early diagnosis and monitoring are important for managing the condition.

Ebstein Anomaly is a congenital malformation characterized by apical displacement of the septal and posterior leaflets of the tricuspid valve, resulting in atrialization of a portion of the right ventricle. This leads to tricuspid regurgitation and right atrial enlargement. The defect arises from abnormal development of the endocardial cushions during embryogenesis. It is often associated with an atrial septal defect or patent foramen ovale, facilitating right-to-left shunting and systemic desaturation. Clinically, it presents with signs of right heart failure, cyanosis, and arrhythmias. The severity of valve displacement and regurgitation determines the clinical course and prognosis.