2026 ICD-10-CM Diagnosis Code Q22.0Pulmonary valve atresia

ICD-10-CM Codes›Q00-Q99›Q20-Q28›Q22

ICD-10-CM Q22.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q22.0 is a billable ICD-10-CM diagnosis code for pulmonary valve atresia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 306 through 307. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Cardiac and circulatory congenital anomalies.

Code Identity

ICD-10-CM Code
Q22.0
Billable Status
Yes — Valid for Submission
Code Describes
Pulmonary valve atresia
Short Description
Pulmonary valve atresia
Same as the full description in the CMS dataset.
Parent Code
Q22
Congenital malformations of pulmonary and tricuspid valves

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ20-Q28Congenital malformations of the circulatory system
CategoryQ22Congenital malformations of pulmonary and tricuspid valves
This CodeQ22.0Pulmonary valve atresia

Present on Admission (POA)Billing

Q22.0 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Absence of pulmonary valve cusp
  • Absent pulmonary valve syndrome
  • Absent pulmonary valve syndrome with ventricular septal defect of non Fallot type
  • Agenesis of artery
  • Atresia of pulmonary valve
  • Congenital abnormality of thoracic aorta and pulmonary arteries
  • Congenital absence of ductus arteriosus
  • Congenital absence of pulmonary valve
  • Congenital atresia of pulmonary valve
  • Congenital hypoplasia of pulmonary valve
  • Hypoplasia of pulmonary cusps of absent pulmonary valve type
  • Hypoplasia of pulmonary valve
  • Muscular pulmonary atresia
  • Patent ductus arteriosus
  • Pulmonary atresia and ventricular septal defect with aorta from left ventricle
  • Pulmonary atresia and ventricular septal defect with aorta from right ventricle
  • Pulmonary atresia with intact ventricular septum
  • Pulmonary atresia with ventricular septal defect
  • Pulmonary atresia with ventricular septal defect of Fallot type
  • Pulmonary valve agenesis, intact ventricular septum, persistent ductus arteriosus syndrome
  • Pulmonary valve agenesis, tetralogy of Fallot, absence of ductus arteriosus syndrome
  • Pulmonary valve atresia without ventricular outflow tract
  • Pulmonary valve cusp hypoplasia
  • Tetralogy of Fallot
  • Tetralogy of Fallot with absent pulmonary valve
  • Tetralogy of Fallot with atresia of pulmonary valve
  • Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral artery

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Absence(of) (organ or part) (complete or partial)
      • pulmonary valve
    • Agenesis
      • heart
        • valve NEC
          • pulmonary
    • Anomaly, anomalous(congenital) (unspecified type)
      • heart
        • valve NEC
          • pulmonary
            • atresia
    • Anomaly, anomalous(congenital) (unspecified type)
      • pulmonary
        • valve
          • atresia
    • Atresia, atretic
      • heart valve NEC
        • pulmonary
    • Atresia, atretic
      • pulmonary (artery)
        • valve
    • Atresia, atretic
      • pulmonic

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL001
Cardiac and circulatory congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • 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.

Patient EducationClinical

Congenital Heart Defects

Congenital heart defects (CHDs) are problems with the structure of the heart. "Congenital" means that that the problems are present at birth. These defects happen when a fetus's heart doesn't develop normally during pregnancy. Congenital heart defects are the most common type of birth defect.

The full article covers:

  • What are congenital heart defects?
  • What causes congenital heart defects?
  • Who is more likely to have a baby with a congenital heart defect?
  • What are the symptoms of congenital heart defects?
  • What other problems do congenital heart defects cause?
  • How are congenital heart defects diagnosed?
  • What are the treatments for congenital heart defects?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q22.0 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
746.01 Cong pulmon valv atresia
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q22.0Overview

Is Q22.0 (Congenital malformations of pulmonary and tricuspid valves) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report pulmonary valve atresia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q22.0 group to?

When pulmonary valve atresia is the principal diagnosis on an inpatient stay, it groups to MS-DRG 306, 307, with relative weights from 0.9132 to 1.5758 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q22.0 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for pulmonary valve atresia on inpatient claims.

What is the ICD-9 equivalent of Q22.0?

Under the General Equivalence Mappings, pulmonary valve atresia converts to ICD-9-CM 746.01 (cong pulmon valv atresia). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.