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IPCCC ICD-11 Congenital Heart Atlas

You are here: Ebstein malformation of tricuspid valve Congenital anomaly of tricuspid valve Congenital anomaly of an atrioventricular valve or atrioventricular septum Structural developmental anomaly of heart or great vessels

IPCCC Term Ebstein malformation of tricuspid valve
IPCCC Code 06.01.34
ICD-11 MMS LA87.03
ICD-11 Code 307157712

Definition

A congenital cardiac malformation of the tricuspid valve and right ventricle that is characterized 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.

Synonyms/Abbreviations

Ebstein syndrome; Ebstein anomaly; Ebstein disease; Ebstein anomaly of tricuspid valve; Ebstein's malformation of tricuspid valve; Ebstein; anomaly or syndrome; Tricuspid valve, Ebstein anomaly; Congenital Ebstein deformity of tricuspid valve

STS-EACTS-derived IPCCC term

Tricuspid valve disease, Ebstein's anomaly

EPCC-derived IPCCC term

Ebstein malformation of tricuspid valve

ICD-11 MMS code or crossmap

LA87.03

ICD-10 MMS code or crossmap

Q22.5: Ebstein anomaly

Parent

Congenital anomaly of tricuspid valve

Siblings

Coding Notes

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 the great arteries], Ebstein malformation of tricuspid valve may be present.

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