2027 ICD-10-CM Diagnosis Code Q22.1Congenital pulmonary valve stenosis

ICD-10-CM Codes›Q00-Q99›Q20-Q28›Q22

ICD-10-CM Q22.1
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q22.1 is a billable ICD-10-CM diagnosis code for congenital pulmonary valve stenosis. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 306 through 307. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 62 closely related codes. 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
Q22.1
Billable Status
Yes — Valid for Submission
Code Describes
Congenital pulmonary valve stenosis
Short Description
Congenital pulmonary valve stenosis
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 CodeQ22.1Congenital pulmonary valve stenosis

Present on Admission (POA)Billing

Q22.1 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Congenital malformations of the circulatory system (Q20-Q28).

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Arterial duct dependent pulmonary circulation
  • Congenital critical stenosis of pulmonary valve with ductus arteriosus dependent pulmonary circulation
  • Congenital stenosis of pulmonary valve
  • Double outlet right ventricle
  • Double outlet right ventricle with doubly committed ventricular septal defect
  • Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis
  • Double outlet right ventricle with subaortic or doubly committed ventricular septal defect and pulmonary stenosis Fallot type
  • Double outlet right ventricle with subaortic ventricular septal defect
  • Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis Fallot type
  • Doubly committed subarterial ventricular septal defect
  • Tetralogy of Fallot
  • Tetralogy of Fallot with pulmonary stenosis

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q22.1: its own notes plus those printed at Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q22.1, its category, or a range that includes it.

Excludes1 1

These codes carry an Excludes1 note naming Q22.1: they are not reported together with it, unless the two conditions are unrelated.

  • I37 Nonrheumatic pulmonary valve disorders
    pulmonary valve disorder specified as congenital (Q22.1, Q22.2, Q22.3)

Excludes2 1

These codes carry an Excludes2 note naming Q22.1: its condition is not part of those codes, and both may be reported when the patient has both.

  • I08 Multiple valve diseases
    multiple valve disease specified as nonrheumatic (I34.-, I35.-, I36.-, I37.-, I38.-, Q22.-, Q23.-, Q24.8-) names Q22.-, which includes this code

Use Additional Code 1

These codes say to use an additional code from the note, and Q22.1 is one of the codes named.

  • Q87.88 CTNNB1 syndrome
    congenital heart malformations (Q20.0-Q24.9) names Q20.0-Q24.9, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

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

  • Congenital Pulmonary Valve Stenosis

    pulmonary valve stenosis that is present at birth.

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 Q22.1 to ICD-9-CMHistory

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

ICD-9-CM
746.02 Cong pulmon valve stenos
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 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q22.1Overview

What is the ICD-10 code for congenital pulmonary valve stenosis?

The ICD-10-CM code for congenital pulmonary valve stenosis is Q22.1 (sometimes written as Q221). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q22.1 (Congenital malformations of pulmonary and tricuspid valves) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report congenital pulmonary valve stenosis on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does Q22.1 group to?

When congenital pulmonary valve stenosis is the principal diagnosis on an inpatient stay, it groups to MS-DRG 306, 307, with relative weights from 0.9146 to 1.5457 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q22.1 a CC or MCC?

CMS lists Q22.1 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 62 closely related codes in its exclusion list.

Is Q22.1 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 congenital pulmonary valve stenosis on inpatient claims. The code appears in the Congenital malformations of the circulatory system (Q20-Q28) range of the CMS exempt list.