Supravalvular aortic stenosis

This code identifies a congenital narrowing of the aorta just above the aortic valve. This malformation restricts blood flow from the left ventricle to the rest of the body, leading to increased workload on the heart. It is a distinct anatomical anomaly from stenosis occurring at or below the valve.

Assign this code when documentation specifically indicates supravalvular aortic stenosis, a congenital condition. This diagnosis is typically made through echocardiography or cardiac catheterization, confirming the location of the narrowing above the aortic valve. Use this code for both initial diagnosis and subsequent encounters for management of this specific congenital defect.

• Differentiate carefully between supravalvular, valvular, and subvalvular aortic stenosis based on physician documentation.
• Do not code this with Q23.0 (congenital aortic stenosis NOS or congenital stenosis of aortic valve) due to the Excludes1 note.
• Ensure the documentation clearly specifies the supravalvular location to avoid miscoding with other forms of congenital aortic stenosis.