Ebstein malformation of the tricuspid valve

Orpha code: 1880OMIM code: 224700

Definicja

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.

Disease data
Klasyfikacja

Morphological anomaly

Synonimy
Ebstein anomaly of the tricuspid valve
Anomalia Ebsteina zastawki trójdzielnej
Kod ORPHA
1880
Kod OMIM
224700
Kod ICD10
Q22.5
Kod ICD11
LA87.03

No additional description.

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