Ebstein anomaly

Ebstein anomaly is a rare form of cyanotic congenital heart disease characterised by a downwards displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle. This displaces the "border" between the right atrium and right ventricle inferiorly, making the right atrium larger and right ventricle smaller, a process called atrialisation.

Depending on the exact pathology and any concomitant heart defects, the clinical features can vary significantly.

Diagnosis and evaluation

Echocardiography confirms the diagnosis. Chest radiography shows a box-shaped heart.

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