Ebstein's anomaly

This code represents a rare congenital heart defect characterized by the apical displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle. This displacement leads to atrialization of a portion of the right ventricle, tricuspid regurgitation, and often right atrial enlargement. The severity of the anomaly is highly variable, ranging from mild forms with minimal symptoms to severe forms presenting with cyanosis and heart failure in infancy.

Use this code for patients diagnosed with Ebstein's anomaly, regardless of the severity or presence of associated conditions. This code is appropriate when the medical record clearly documents the diagnosis of Ebstein's anomaly, often confirmed by echocardiography or other cardiac imaging. It should be applied for both initial diagnoses and subsequent encounters related to the management of this congenital defect.

• Always code any associated congenital heart defects, such as atrial septal defect (ASD) or patent foramen ovale (PFO), separately.
• Specify any manifestations or complications, such as heart failure (I50.-) or arrhythmias (I47-I49), with additional codes.
• Ensure the documentation clearly distinguishes Ebstein's anomaly from other forms of tricuspid valve abnormalities.