2027 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 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q87.40 is a billable ICD-10-CM diagnosis code for marfan syndrome, unspecified. 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 49 closely related codes. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Q87.40 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 84 for payment year 2027.

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 the other POA exempt codes in Other congenital malformations (Q80-Q89).

Medicare Risk Adjustment (HCC)Billing

Q87.40 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model, but it maps to payment categories in the other CMS models shown below.

CMS-HCC V28 (Medicare Advantage Payment Model)
Not mapped
no payment category in the model that pays 100% of MA risk scores
Prior Model (CMS-HCC V24)
Not mapped under V24
this diagnosis newly risk-adjusts under the V28 model
Part D (RxHCC)
RxHCC 84 — Systemic Lupus Erythematosus and Other Systemic Connective Tissue Disorders
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

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

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q87.40: its own notes plus those printed at Q87 and 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)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

From Q87 Other specified congenital malformation syndromes affecting multiple systems applies to 21 codes
  • code(s) to identify all associated manifestations

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 Q87.40, its category, or a range that includes it.

Excludes1 6

These codes carry an Excludes1 note naming Q87.40: they are not reported together with it, unless the two conditions are unrelated.

  • Q87.A Loeys-Dietz syndrome
    Marfan syndrome (Q87.40-Q87.43)
  • Block E70-E88 Metabolic disorders
    Marfan syndrome (Q87.4-) names Q87.4-, which includes this code
  • I34.1 Nonrheumatic mitral (valve) prolapse
    Marfan's syndrome (Q87.4-) names Q87.4-, which includes this code
  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q75 Other congenital malformations of skull and face bones
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q89.7 Multiple congenital malformations, not elsewhere classified
    congenital malformation syndromes affecting multiple systems (Q87.-) names Q87.-, which includes this code

Code First 1

These codes say to code first a condition named in the note, and Q87.40 is one of the conditions named.

  • J93.12 Secondary spontaneous pneumothorax
    Marfan syndrome (Q87.4-) names Q87.4-, 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 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 2025–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q87.40Overview

What is the ICD-10 code for marfan syndrome, unspecified?

The ICD-10-CM code for marfan syndrome, unspecified is Q87.40 (sometimes written as Q8740). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

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, 2026 through September 30, 2027.

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 0.9146 to 1.5457 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q87.40 a CC or MCC?

CMS lists Q87.40 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 49 closely related codes in its exclusion list.

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. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.

Does Q87.40 risk-adjust for Medicare Advantage payment?

Not for Medicare Advantage: Q87.40 maps to no category in the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 84 (Systemic Lupus Erythematosus and Other Systemic Connective Tissue Disorders).