Ebstein malformation of the tricuspid valve

Get in touch with RARE Concierge.

Contact RARE Concierge

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 June 2024

Newly diagnosed with
Ebstein malformation of the tricuspid valve?

Our RARE Concierge Services Guides are available to assist you by providing information, resources and connections as you navigate your rare disease journey.

Get Concierge Help

Clinical Trials

For a list of clinical trials in this disease area, please click here.