Skip to main content

Q87.43 ICD-10-CM Code: Marfan syndrome with skeletal manifestation

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

Coding at a Glance

Tabular directives
1 use-additional code
Risk adjustment
RxHCC V08 category 84

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.

  • MS-DRG 306 — CARDIAC CONGENITAL AND VALVULAR DISORDERS WITH MCC (MDC 05)
  • MS-DRG 307 — CARDIAC CONGENITAL AND VALVULAR DISORDERS WITHOUT MCC (MDC 05)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q87.43 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 Q87.43 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 Q87.43

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

Before you code Q87.43

  1. Q87.43’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 →

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 Q87.43(1 note)

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with Q87.43 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 Q87.43 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 Q87.43?

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

Marfan syndrome with skeletal manifestation is a billable ICD-10-CM diagnosis code (Q87.43).

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

  • CC as a secondary diagnosis (FY2026). Can raise the stay's MS-DRG severity tier.

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 Q87.43 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.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 48 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 2 MS-DRGs: DRG 306 (MDC 05), DRG 307 (MDC 05).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):MAL010 — Other specified and unspecified congenital anomalies (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

Q85.89 — Other phakomatoses, not elsewhere classified, Q85.9 — Phakomatosis, unspecified, Q87.11 — Prader-Willi syndrome, Q87.19 — Other congenital malformation syndromes predominantly associated with short stature, Q87.2 — Congenital malformation syndromes predominantly involving limbs, Q87.3 — Congenital malformation syndromes involving early overgrowth, Q87.40 — Marfan syndrome, unspecified, Q87.410 — Marfan syndrome with aortic dilation, Q87.418 — Marfan syndrome with other cardiovascular manifestations, Q87.42 — Marfan syndrome with ocular manifestations, Q87.5 — Other congenital malformation syndromes with other skeletal changes, Q87.81 — Alport syndrome, Q87.82 — Arterial tortuosity syndrome, Q87.83 — Bardet-Biedl syndrome, Q87.84 — Laurence-Moon syndrome, Q87.85 — MED13L syndrome, Q87.86 — Kleefstra syndrome, Q87.87 — Hao-Fountain Syndrome, Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified, Q89.7 — Multiple congenital malformations, not elsewhere classified, +27 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified congenital anomalies).

Q87.0 — Congenital malformation syndromes predominantly affecting facial appearance, Q87.1 — Congenital malformation syndromes predominantly associated with short stature, Q87.11 — Prader-Willi syndrome, Q87.19 — Other congenital malformation syndromes predominantly associated with short stature, Q87.2 — Congenital malformation syndromes predominantly involving limbs, Q87.3 — Congenital malformation syndromes involving early overgrowth, Q87.40 — Marfan syndrome, unspecified, Q87.410 — Marfan syndrome with aortic dilation, Q87.418 — Marfan syndrome with other cardiovascular manifestations, Q87.42 — Marfan syndrome with ocular manifestations, Q87.5 — Other congenital malformation syndromes with other skeletal changes, Q87.81 — Alport syndrome, Q87.82 — Arterial tortuosity syndrome, Q87.83 — Bardet-Biedl syndrome, Q87.84 — Laurence-Moon syndrome, Q87.85 — MED13L syndrome, Q87.87 — Hao-Fountain Syndrome, Q87.88 — CTNNB1 syndrome, Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified, Q89.01 — Asplenia (congenital), +59 more

Contextual Map

Every relationship of Q87.43 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 Q87.43 with these 9 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes (21)

Referenced by Code First instructions

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 05 — Diseases and Disorders of the Circulatory System[MDC crossing]: “Diseases and Disorders of the Circulatory System — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 17,209 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries

  • Syndrome, Marfan, with, skeletal manifestations[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (25)

Change history (3)

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
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 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. "Q87.43 — Marfan syndrome with skeletal manifestation." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/q87.43-marfan-syndrome-with-skeletal-manifestation

Change history

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

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q87.43 in its code family, with their registry titles.

View all codes in the Q87 family