Atresia of pulmonary artery

This code represents a congenital heart defect characterized by the complete absence of the pulmonary artery, preventing blood flow from the right ventricle to the lungs. It is a severe form of congenital heart disease often associated with other cardiac anomalies.

Use this code when documentation clearly states a diagnosis of pulmonary artery atresia. This diagnosis is typically made prenatally or shortly after birth through echocardiography and cardiac catheterization. Supporting documentation should detail the anatomical absence of the pulmonary artery.

• Differentiate from pulmonary artery stenosis, which involves narrowing rather than complete absence.
• Always code any associated congenital heart defects, such as ventricular septal defect (VSD) or patent ductus arteriosus (PDA), as secondary diagnoses.
• Ensure documentation specifies "atresia" to avoid confusion with hypoplasia or severe stenosis.