Q22.0

Billable

Pulmonary valve atresia

HCC Category Mapping

RxHCCHCC 191 — Congenital Heart/Circulatory Disorders and Fontan Circulation

0.000

What This Code Means

A birth defect where the pulmonary valve is completely closed or absent, preventing blood flow from the right ventricle to the lungs.

Coding Tips

•This is a severe condition often requiring surgical intervention; look for associated codes for procedures or complications
•May be associated with other cardiac defects; review for additional diagnoses

Clinical Significance

Pulmonary valve atresia represents complete absence or closure of the pulmonary valve, preventing normal blood flow to the lungs and requiring immediate intervention for survival. This condition necessitates complex surgical procedures to establish pulmonary circulation and requires lifelong cardiac management.

Documentation Requirements

✓Echocardiographic confirmation of absent or imperforate pulmonary valve
✓Assessment of right ventricular development and function
✓Evaluation of pulmonary artery size and development
✓Associated cardiac anomalies
✓Surgical intervention history and type of repair
✓Current oxygen saturation and cyanosis degree
✓Conduit function and need for replacement
✓Exercise tolerance and functional capacity

Commonly Confused Codes

Q22.1 — congenital pulmonary valve stenosis Q25.5 — atresia of pulmonary artery Q21.3 — tetralogy of Fallot with pulmonary atresia Q22.2 — congenital pulmonary valve insufficiency I37.0 — pulmonary valve stenosis (acquired)

Code Hierarchy

└Q22Congenital malformations of pulmonary and tricuspid valves └Q22.0Pulmonary valve atresia

└Q22.0Pulmonary valve atresia