2026 ICD-10-CM Diagnosis Code Q25.3Supravalvular aortic stenosis

ICD-10-CM Codes›Q00-Q99›Q20-Q28›Q25

ICD-10-CM Q25.3
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q25.3 is a billable ICD-10-CM diagnosis code for supravalvular aortic stenosis. 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
Q25.3
Billable Status
Yes — Valid for Submission
Code Describes
Supravalvular aortic stenosis
Short Description
Supravalvular aortic stenosis
Same as the full description in the CMS dataset.
Parent Code
Q25
Congenital malformations of great arteries

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ20-Q28Congenital malformations of the circulatory system
CategoryQ25Congenital malformations of great arteries
This CodeQ25.3Supravalvular aortic stenosis

Present on Admission (POA)Billing

Q25.3 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.

  • Atresia and stenosis of aorta
  • Coarctation of aortic arch
  • Congenital stenosis of aorta
  • Congenital supravalvular aortic stenosis
  • Postductal aortic stenosis
  • Postductal coarctation of aorta
  • Preductal aortic stenosis
  • Preductal coarctation of aorta
  • Stenosis of thoracic aorta
  • Supravalvar aortic stenosis

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Type 1 Excludes

  • congenital aortic stenosis NOS Q23.0
  • congenital stenosis of aortic valve Q23.0

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.

    • Malformation(congenital)
      • aorta
        • stenosis
          • supravalvular
    • Stenosis, stenotic(cicatricial)
      • aorta (ascending) (supraventricular) (congenital)
        • supravalvular
    • Stricture
      • aorta (ascending) (congenital)
        • supravalvular, congenital

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

  • ELN wt Allele|Elastin (Supravalvular Aortic Stenosis, Williams-Beuren Syndrome) Gene|Elastin wt Allele|FLJ38671|FLJ43523|SVAS|WBS|WS

    human eln wild-type allele is located in the vicinity of 7q11.23 and is approximately 42 kb in length. this allele, which encodes elastin protein, plays a role in the regulation of the structure of the extracellular matrix. mutations in the gene are associated with autosomal dominant cutis laxa and supravalvular aortic stenosis. haploinsufficiency may be a factor in williams-beuren syndrome.

  • Supravalvular Aortic Stenosis

    a rare, congenital or acquired aortic stenosis characterized by localized or diffuse narrowing of the ascending aorta.

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 Q25.3 to ICD-9-CMHistory

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

ICD-9-CM
747.22 Aortic atresia/stenosis
Approximate The match is approximate rather than exact.

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 Q25.3Overview

Is Q25.3 (Congenital malformations of great arteries) a billable code?

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

What MS-DRG does Q25.3 group to?

When supravalvular aortic stenosis 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 Q25.3 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 supravalvular aortic stenosis on inpatient claims.

What is the ICD-9 equivalent of Q25.3?

Under the General Equivalence Mappings, supravalvular aortic stenosis converts to ICD-9-CM 747.22 (aortic atresia/stenosis). The mapping is approximate, so confirm the match fits the documentation.

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.