2026 ICD-10-CM Diagnosis Code Q87.40Marfan syndrome, unspecified

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q87

ICD-10-CM Q87.40
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q87.40 is a billable ICD-10-CM diagnosis code for marfan syndrome, unspecified. 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 Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q87.40
Billable Status
Yes — Valid for Submission
Code Describes
Marfan syndrome, unspecified
Short Description
Marfan syndrome, unspecified
Same as the full description in the CMS dataset.
Parent Code
Q87.4
Marfan syndrome

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ87Other specified congenital malformation syndromes affecting multiple systems
This CodeQ87.40Marfan syndrome, unspecified

Present on Admission (POA)Billing

Q87.40 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.

  • Blindness, scoliosis, arachnodactyly syndrome
  • Congenital anomaly of subcutaneous tissue
  • Congenital inguinal hernia
  • Congenital nephritis
  • Marfan syndrome type 1
  • Marfanoid facies
  • Marfanoid habitus with autosomal recessive intellectual disability syndrome
  • Marfanoid habitus, inguinal hernia, advanced bone age syndrome
  • Marfanoid physique
  • Marfan's syndrome
  • Marfan's syndrome affecting skin
  • Microcephalus, glomerulonephritis, marfanoid habitus syndrome
  • Neonatal Marfan syndrome
  • Painful orbital and systemic neurofibroma, marfanoid habitus syndrome
  • Plexiform neurofibroma
  • Progeroid and marfanoid aspect, lipodystrophy syndrome
  • X-linked intellectual disability with marfanoid habitus

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.

    • Syndrome
      • Beals
    • Syndrome
      • Marfan

Clinical ClassificationClinical

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

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Marfan Syndrome

    an autosomal dominant disorder of connective tissue with abnormal features in the heart, the eye, and the skeleton. cardiovascular manifestations include mitral valve prolapse; aortic aneurysm; and aortic dissection. other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged dura mater (dural ectasia). marfan syndrome (type 1) is associated with mutations in the gene encoding fibrillin-1 (fbn1), a major element of extracellular microfibrils of connective tissue. mutations in the gene encoding type ii tgf-beta receptor (tgfbr2) are associated with marfan syndrome type 2.

Patient EducationClinical

Marfan Syndrome

Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, and other organs. One of these proteins is fibrillin. A problem with the fibrillin gene causes Marfan syndrome.

Read the full article at MedlinePlus

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

Convert Q87.40 to ICD-9-CMHistory

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

ICD-9-CM
759.82 Marfan syndrome
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 2017–2023No changes
FY 2024RevisedCode revised
FY 2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q87.40Overview

Is Q87.40 (Marfan syndrome) a billable code?

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

What MS-DRG does Q87.40 group to?

When marfan syndrome, unspecified 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 Q87.40 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 marfan syndrome, unspecified on inpatient claims.

What is the ICD-9 equivalent of Q87.40?

Under the General Equivalence Mappings, marfan syndrome, unspecified converts to ICD-9-CM 759.82 (marfan syndrome). 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.