Pulmonary infundibular stenosis

This code represents a congenital heart defect characterized by a narrowing of the infundibulum, the muscular outflow tract of the right ventricle leading to the pulmonary artery. This obstruction impedes blood flow from the right ventricle to the lungs, increasing pressure within the right ventricle. The severity can range from mild to severe, impacting the degree of right ventricular hypertrophy and potential cyanosis.

Use this code when documentation explicitly states a diagnosis of pulmonary infundibular stenosis. This typically occurs in pediatric cardiology settings following diagnostic imaging such as echocardiography, cardiac MRI, or cardiac catheterization. The code is appropriate for both initial diagnosis and subsequent encounters for management of this specific congenital anomaly.

• Differentiate from pulmonary valve stenosis (Q22.1) or supravalvular pulmonary stenosis (Q24.8), as these are distinct anatomical locations of obstruction.
• Always review imaging reports and cardiologist notes to confirm the precise location and nature of the pulmonary outflow tract obstruction.
• Consider sequencing this code as a primary diagnosis when it is the main reason for the encounter, or as a secondary diagnosis when managing other related congenital heart defects.