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Q87.41 ICD-10-CM Code: Marfan syndrome with cardiovascular manifestations

Compare with another code for Q87.41Check this code on a claim

Billing Status: NO. This is a non-billable ICD-10-CM code: report a more specific billable code beneath it.

Coding at a Glance

Official instructions
1 use-additional instruction

What these terms mean →

Not billable · FY2027A non-billable heading in the tabular list: report a more specific code beneath it.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too, and is marked with where it comes from.

Use additional codefrom Q87Report with this code when documented
  • Use additional code(s) to identify all associated manifestations

Where it sits Source: CMS/NCHS

Under:Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-QA1) (Q00-QA1)Q80-Q89 Other congenital malformationsQ87 Other specified congenital malformation syndromes affecting multiple systemsQ87.4 Marfan syndrome

Codes under Q87.41 (2)

  • Q87.410Marfan syndrome with aortic dilationBillable
  • Q87.418Marfan syndrome with other cardiovascular manifestationsBillable

CompareCheck ClaimView Related Codes

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q87.41 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on Q87.41 itself; “inherited from” names the category or block whose note applies here.

Use Additional Code

Supplementary codes the tabular list directs you to add.

  • Use additional code(s) to identify all associated manifestations

Source: inherited from Q87

Coder workflow for Q87.41

MedCoder structured workflow — derived from this code’s own official record

Before you code Q87.41

  1. Q87.41 is not reportable as written. Select the more specific code beneath it that the documentation supports. Codes are reported to the highest level of specificity the classification provides (Guidelines I.B.2).

    ReviewQ87.410, Q87.418

    See the relationships section · Guide: How to choose an ICD-10-CM code →

  2. Q87.41’s title joins a condition with an associated condition or complication. Confirm each component is documented. Where the classification presumes the link through the “with” convention, only a provider statement that the conditions are unrelated defeats it. A combination code is assigned only when it fully identifies the documented conditions; a required second code for the stage, type or manifestation is still reported when the notes ask for it (Guidelines I.B.9, I.A.15).

    Guide: Combination codes →

Choose the right path

  1. Does the documentation support one of the more specific codes beneath Q87.41?
    Yes → Select that code and continue the checks below on its own page.
    No → Q87.41 cannot be reported as written. Where the codes beneath it include an unspecified code, that code applies when the record goes no further; otherwise query for the missing detail.

    ReviewQ87.410, Q87.418

Report the code beneath Q87.41 that the documentation supports, not Q87.41 itself. Then work the Use Additional Code note, and confirm that code is valid for the date of service on its own page.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
The associated condition or complication
Whether the associated condition the title names is documented; the “with” convention presumes some links, and a provider statement that the conditions are unrelated defeats it (Guidelines I.A.15).
The conditions the Use Additional Code note names
Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.

Official instructions as workflow

  • Use Additional Code — after identifying Q87.41(1 note)

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with Q87.41 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.

    See the official tabular notes · Guidelines I.A.13

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Only one of the components this code’s title joins is documented.

Coding question: Is the code beneath Q87.41 supported?

Path: Review the code for the documented component on its own.

Reason: A combination code is assigned only when it fully identifies the documented conditions; otherwise the documented component takes its own code (Guidelines I.B.9).

Documentation: A condition the Use Additional Code note names is documented.

Coding question: Is a second code reported with the code beneath Q87.41?

Path: Review the Use Additional Code note and the code it names.

Reason: The additional code is reported when the record documents the condition; a conditional instruction applies only when its condition is met (Guidelines I.A.13).

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Don’t confuse Q87.41 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of Q87.41 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • Q87.40— Specificity relationship (Review)

    Overlapping diagnoses in the same category.

    Overlapping codes in the same category — the documentation decides which is the more specific fit.

    What moves you there: A record that documents no more than “Marfan syndrome, unspecified” takes Q87.40; specificity in the record moves it back here.

    Full comparison: Q87.41 vs Q87.40 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Marfan syndrome with cardiovascular manifestations is a non-billable ICD-10-CM category code (Q87.41). A more specific billable subcode must be selected for claims submission.

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Verify Before Coding

  • Not billable as written — a more specific code is required: Q87.410, Q87.418.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name Q87.41 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 21 Excludes1 notes across 3 chapters: E70 — Disorders of aromatic amino-acid metabolism (via Q87.4.-), E70-E88 — Metabolic disorders (E70-E88) (via Q87.4.-), E71 — Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism (via Q87.4.-), E72 — Other disorders of amino-acid metabolism (via Q87.4.-), E73 — Lactose intolerance (via Q87.4.-), E74 — Other disorders of carbohydrate metabolism (via Q87.4.-), E75 — Disorders of sphingolipid metabolism and other lipid storage disorders (via Q87.4.-), E76 — Disorders of glycosaminoglycan metabolism (via Q87.4.-), E77 — Disorders of glycoprotein metabolism (via Q87.4.-), E78 — Disorders of lipoprotein metabolism and other lipidemias (via Q87.4.-), E79 — Disorders of purine and pyrimidine metabolism (via Q87.4.-), E80 — Disorders of porphyrin and bilirubin metabolism (via Q87.4.-), E83 — Disorders of mineral metabolism (via Q87.4.-), E84 — Cystic fibrosis (via Q87.4.-), E85 — Amyloidosis (via Q87.4.-), E86 — Volume depletion (via Q87.4.-), E87 — Other disorders of fluid, electrolyte and acid-base balance (via Q87.4.-), E88 — Other and unspecified metabolic disorders (via Q87.4.-), I34.1 — Nonrheumatic mitral (valve) prolapse (via Q87.4.-), Q87.A — Loeys-Dietz syndrome, +1 more.

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Referenced by 1 Code First instruction: J93.12 — Secondary spontaneous pneumothorax (via Q87.4.-).

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

Contextual Map

Every relationship of Q87.41 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run Q87.41 with these 9 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes (21)

Referenced by Code First instructions

Nearest codes (25)

Change history (3)

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Common coding questions

MedCoder editorial

Can Q87.41 be billed directly?

No. Q87.41 (Marfan syndrome with cardiovascular manifestations) is a non-billable ICD-10-CM category code. A more specific billable subcode must be selected based on clinical documentation.

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 2026) Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder-derived relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder editorial explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "Q87.41 — Marfan syndrome with cardiovascular manifestations." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/q87.41-marfan-syndrome-with-cardiovascular-manifestations

Change history

  • FY2024 — October 1, 2023
    Description revised
    Marfan's syndrome with cardiovascular manifestations → Marfan syndrome with cardiovascular manifestations
    FY2024 changes
  • FY2024 — October 1, 2023
    Short description revised
    Marfan's syndrome with cardiovascular manifestations → Marfan syndrome with cardiovascular manifestations
    FY2024 changes
  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Marfan's syndrome with cardiovascular manifestations

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q87.41 in its code family, with their registry titles.

View all codes in the Q87 family