Pulmonary valve atresia

This code represents a congenital heart defect where the pulmonary valve, which controls blood flow from the right ventricle to the pulmonary artery, is completely absent or imperforate. This obstruction prevents blood from reaching the lungs for oxygenation, leading to severe cyanosis and requiring immediate intervention.

Assign this code for documented cases of pulmonary valve atresia identified at birth or prenatally. Supporting documentation includes echocardiogram reports, cardiac catheterization findings, and physician notes explicitly stating the diagnosis of pulmonary atresia.

• Differentiate from pulmonary stenosis, where the valve is narrowed but not completely absent.
• Always code any associated cardiac anomalies, such as ventricular septal defect (VSD) or patent ductus arteriosus (PDA), which are often present.
• Consider sequencing this code as the primary diagnosis when it is the main reason for the encounter or admission.