Congenital malformations of pulmonary and tricuspid valves (Q22) ICD-10-CM
The Q22 code range covers congenital malformations of pulmonary and tricuspid valves with 10 ICD-10-CM diagnosis codes. 9 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Codes in the Q22 Range 10 codes · 9 billable
- Q22 Congenital malformations of pulmonary and tricuspid valvesNon-billable
- Q22.0 Pulmonary valve atresia
- Q22.1 Congenital pulmonary valve stenosis
- Q22.2 Congenital pulmonary valve insufficiency
- Q22.3 Other congenital malformations of pulmonary valve
- Q22.4 Congenital tricuspid stenosis
- Q22.5 Ebstein's anomaly
- Q22.6 Hypoplastic right heart syndrome
- Q22.8 Other congenital malformations of tricuspid valve
- Q22.9 Congenital malformation of tricuspid valve, unspecified
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the Q22 range.
Double Outlet Right Ventricle
Incomplete transposition of the great vessels in which both the AORTA and the PULMONARY ARTERY arise from the RIGHT VENTRICLE. The only outlet of the LEFT VENTRICLE is a large ventricular septal defect (VENTRICULAR SEPTAL DEFECTS or VSD). The various subtypes are classified by the location of the septal defect, such as subaortic, subpulmonary, or noncommitted.
Tetralogy of Fallot
A combination of congenital heart defects consisting of four key features including VENTRICULAR SEPTAL DEFECTS; PULMONARY STENOSIS; RIGHT VENTRICULAR HYPERTROPHY; and a dextro-positioned AORTA. In this condition, blood from both ventricles (oxygen-rich and oxygen-poor) is pumped into the body often causing CYANOSIS.
About the Q22 Code Range
ICD-10 code Q22 covers congenital malformations involving the pulmonary and tricuspid heart valves. These codes are specifically used to identify and classify birth defects of these heart valves, including conditions such as pulmonary valve atresia, tricuspid stenosis, and Ebstein's anomaly.
The section begins with Q22.0 for pulmonary valve atresia, which is also known as congenital atresia of the pulmonary valve or hypoplasia of the pulmonary valve. This code is essential for capturing complex defects like Tetralogy of Fallot with pulmonary valve issues. Q22.1 denotes congenital pulmonary valve stenosis, often linked with double outlet right ventricle or Tetralogy of Fallot variations. Insufficiency or leaking of the pulmonary valve is coded as Q22.2. Other abnormalities affecting the pulmonary valve, including cusp abnormalities and valve dysplasia, fall under Q22.3. For tricuspid valve issues, congenital tricuspid stenosis (Q22.4) includes Ebstein’s anomaly and valve atresia, while Q22.5 specifically codes for Ebstein’s anomaly itself, which may present with atrial septal defects. Hypoplastic right heart syndrome is identified by Q22.6. Additional congenital problems such as accessory tissues, valve clefts, and chordae abnormalities of the tricuspid valve are classified under Q22.8, and unspecified congenital tricuspid malformations are coded as Q22.9. Using these codes accurately helps medical professionals and coders pinpoint rare pediatric heart valve defects and tailor clinical care and reporting accordingly.
Questions About This Page
How many billable codes are in the Q22 range?
Of the 10 codes in this range, 9 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the Q22 range classify?
The range classifies congenital malformations of pulmonary and tricuspid valves. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
