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Ebstein's anomaly is a rare heart defect in which the tricuspid valve — the valve between the upper right chamber (right atrium) and the lower right chamber (right ventricle) of the heart — doesn't work properly. As a result, blood leaks back through the valve and into the right atrium. Atrial septal defect (labeled patent foramen ovale) is a hole between the two upper chambers of the heart. About half the people with Ebstein's anomaly have an atrial septal defect.