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Q87.A ICD-10-CM Code: Loeys-Dietz syndrome

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
4 Excludes1 · 1 use-additional code

Code Set: New for FY2027 — not valid before October 1, 2026.

Coding instructions

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

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

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

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

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.A

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

Before you code Q87.A

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with Q87.A. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support a condition named in Q87.A’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewQ87.82, Q79.6, M35

Consider Q87.A. Then work the Use Additional Code note, and confirm the code is valid for the date of service in the Verify section.

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

  • Excludes1 — check before selecting Q87.A(4 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with Q87.A: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareQ87.82, Q79.6, M35

    See the official tabular notes · Guidelines I.A.12.a

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

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with Q87.A 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: Both the condition Q87.A describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewQ87.82, Q79.6, M35

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

Coding question: Is a second code reported with Q87.A?

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.

Code Overview

Loeys-Dietz syndrome is a billable ICD-10-CM diagnosis code (Q87.A).

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

Indexed Clinical Terms (1)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

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 structured relationships — computed from published CMS and AHRQ datasets

Other codes that name Q87.A 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 1 Excludes1 note: Q89.7 — Multiple congenital malformations, not elsewhere classified (via Q87.-).

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

Contextual Map

Every relationship of Q87.A 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.A with these 9 related codes in Claim Check

Hierarchy

Excludes1 (9)

Referenced by Excludes1 notes

Index entries

  • Syndrome, Loeys-Dietz[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (25)

Change history

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 FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 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 structured 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 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.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

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.A — Loeys-Dietz syndrome." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/q87.a-loeys-dietz-syndrome

Change history

  • Upcoming · effective FY2027 — October 1, 2026
    Will be added to the code set
    Loeys-Dietz syndrome
    FY2027 changes

Nearest Codes in This Family

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

View all codes in the Q87 family