Congenital mitral stenosis

This code represents a congenital heart defect characterized by a narrowing of the mitral valve, the valve between the left atrium and left ventricle. This stenosis obstructs blood flow from the left atrium to the left ventricle, leading to increased pressure in the left atrium and pulmonary circulation. The condition is present at birth and can vary in severity.

Assign this code when documentation clearly indicates a diagnosis of congenital mitral valve stenosis. This typically occurs in pediatric cardiology settings or during initial evaluations of infants with heart murmurs, cyanosis, or signs of heart failure. Supporting documentation includes echocardiogram reports detailing mitral valve morphology and function, and physician notes confirming the congenital nature of the defect.

• Differentiate from acquired mitral stenosis (I05.0) which develops later in life, often due to rheumatic fever.
• Always review documentation for laterality if specified, though mitral stenosis is typically a singular valve issue.
• Code any associated congenital heart anomalies (e.g., Q21.0 for ventricular septal defect) in addition to this code.