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LA87.03 Ebstein malformation of tricuspid valve

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

synonyms

• Ebstein malformation of tricuspid valve
• Ebstein anomaly
• Ebstein disease
• Accessory tricuspid valve tissue
• congenital Ebstein deformity of tricuspid valve
• Ebstein syndrome
• Ebstein cardiopathy
• Ebstein anomaly of tricuspid valve

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