Synonyms: Ebstein anomaly of the tricuspid valve
A rare congenital cardiac anomaly characterized by downward (apical) displacement of the functional annulus due to incomplete delamination of the septal and inferior leaflets of the tricuspid valve such that they are hinged within the right ventricle rather than as expected at the atrioventricular junction. The anterosuperior leaflet is often abnormal (redundancy fenestrations tethering with abnormal subvalvar apparatus). The atrioventricular junction and the ”atrialized” portion of the right ventricle are dilated with variable degrees of thinning of the right ventricular wall.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version May 2026
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Ebstein malformation of the tricuspid valve?
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Advocacy Organizations
Heart of a Giant Foundation
The mission of the HGF is to achieve better health outcomes for heart diseases and related chronic health conditions in our communities and enhance the quality of life through health education, care support, and connection to community resources.
RareDNA Foundation
Dedicated to improving outcomes for individuals and families affected by rare DNA heart mutations by advancing awareness, access and action. We increase awareness among medical professionals and the public, provide education and resources on diagnosis and treatment, support scientific research, and advocate for faster diagnostic pathways, giving families the knowledge and care they need.
Uganda Alliance of Patients Organization
Supporting patients to access quality, safe and patient-centered healthcare services.
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Clinical Trials
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