Ebstein's anomaly

This code represents Ebstein's anomaly, a rare congenital heart defect characterized by the downward displacement of the tricuspid valve into the right ventricle. This displacement leads to an "atrialized" portion of the right ventricle and often results in tricuspid regurgitation and right heart failure. The severity of the anomaly is highly variable, ranging from mild to life-threatening.

Use this code when documentation explicitly states a diagnosis of Ebstein's anomaly. This diagnosis is typically made through echocardiography, which visualizes the displaced tricuspid valve leaflets and assesses the degree of regurgitation and ventricular dysfunction. Clinical findings such as cyanosis, heart murmur, and arrhythmias in a neonate or child would support this diagnosis.

• Ensure the documentation clearly specifies "Ebstein's anomaly" and not just a generic tricuspid valve abnormality.
• Consider co-occurring congenital heart defects, such as atrial septal defect (ASD), which are frequently associated with Ebstein's anomaly and should be coded separately.
• Sequence this code as the primary diagnosis when it is the main reason for the encounter or hospitalization.