Ebstein malformation of tricuspid valve

Description

A congenital cardiovascular malformation of the tricuspid valve and right ventricle that is characterised by incomplete delamination of the septal and inferior (posterior) tricuspid valvar leaflets from the myocardium of the right ventricle, and varying degrees of downward (apical) rotational displacement of the functional annulus. Additional information: associated cardiac anomalies include an interatrial communication, the presence of accessory conduction pathways and varying degrees of right ventricular outflow tract obstruction, including pulmonary atresia. In the setting of discordant atrioventricular and ventriculo-arterial connections ['Congenitally corrected transposition of great arteries'], 'Ebstein malformation of tricuspid valve' may be present.

Index terms

Ebstein malformation of tricuspid valve
Accessory tricuspid valve tissue
Ebstein anomaly

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Ebstein anomaly of tricuspid valve
Ebstein cardiopathy
Ebstein disease
Ebstein syndrome
congenital Ebstein deformity of tricuspid valve

Exclusions from above levels

LA89.1 – Tricuspid atresia
http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LA87.00 – Congenital tricuspid regurgitation
LA87.01 – Congenital tricuspid valvar stenosis
LA87.02 – Dysplasia of tricuspid valve
LA87.0Y – Other specified congenital anomaly of tricuspid valve
LA87.0Z – Congenital anomaly of tricuspid valve, unspecified

Foundation entity

Ebstein malformation of tricuspid valve – http://id.who.int/icd/entity/307157712

Part of: LA87.0: Congenital anomaly of tricuspid valve › LA87: Congenital anomaly of an atrioventricular valve or atrioventricular septum › 20: Developmental anomalies