Congenital stenosis of aortic valve

This code describes a congenital narrowing or obstruction of the aortic valve, present at birth, which impedes blood flow from the left ventricle to the aorta. This structural defect can range in severity, affecting the leaflets, annulus, or commissures of the valve.

Assign this code when documentation explicitly states congenital stenosis of the aortic valve. This diagnosis is typically made in neonates, infants, or children, often identified during echocardiography or cardiac catheterization. Supporting documentation should detail the presence of a congenital aortic valve anomaly causing obstruction.

• Differentiate from acquired aortic stenosis, which is coded elsewhere.
• Carefully review documentation to exclude cases of hypoplastic left heart syndrome or subaortic/supravalvular stenosis, as these have separate codes.
• Ensure the "congenital" aspect of the aortic valve stenosis is clearly documented to support this specific code.