Skip to main content

I27.841 ICD-10-CM Code: Fontan-associated lymphatic dysfunction

Compare with another code for I27.841Check this code on a claim

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

Coding at a Glance

Official instructions
4 code-also instructions
Risk adjustment
RxHCC V08 category 191

What these terms mean →

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

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.

Code alsoMay be needed with this code
  • associated conditions such as:
  • chylothorax (J94.0)
  • Fontan associated protein-losing enteropathy (K90.49)
  • plastic (obstructive) bronchitis (J44.89)

Where it sits Source: CMS/NCHS

Under:Chapter 9: Diseases of the Circulatory SystemI26-I28 Pulmonary heart disease and diseases of pulmonary circulationI27 Other pulmonary heart diseasesI27.8 Other specified pulmonary heart diseasesI27.84 Fontan related circulation

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

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 v44, Appendix B.

  • MS-DRG 808 — MAJOR HEMATOLOGICAL AND IMMUNOLOGICAL DIAGNOSES EXCEPT SICKLE CELL CRISIS AND COAGULATION DISORDERS WITH MCC (MDC 16)
  • MS-DRG 809 — MAJOR HEMATOLOGICAL AND IMMUNOLOGICAL DIAGNOSES EXCEPT SICKLE CELL CRISIS AND COAGULATION DISORDERS WITH CC (MDC 16)
  • MS-DRG 810 — MAJOR HEMATOLOGICAL AND IMMUNOLOGICAL DIAGNOSES EXCEPT SICKLE CELL CRISIS AND COAGULATION DISORDERS WITHOUT CC/MCC (MDC 16)

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 I27.841 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

Code Also

Additional codes that may be required to fully describe the encounter.

  • associated conditions such as:
  • chylothorax (J94.0)
  • Fontan associated protein-losing enteropathy (K90.49)
  • plastic (obstructive) bronchitis (J44.89)

Coder workflow for I27.841

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

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).

Official instructions as workflow

  • Code Also — related condition(4 notes)

    Coding workflow: Review the related condition when both are documented and the instruction applies. A Code Also note does not fix sequencing; the order follows the circumstances of the encounter.

    ReviewJ94.0, K90.49, J44.89

    See the official tabular notes · Guidelines I.A.17

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 I27.841 with…

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

The codes most likely to be picked instead of I27.841 — 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.

  • I27.849— 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 “Fontan related circulation, unspecified” takes I27.849; specificity in the record moves it back here.

    Full comparison: I27.841 vs I27.849 →

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

Fontan-associated lymphatic dysfunction is a billable ICD-10-CM diagnosis code (I27.841).

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

Official Coding Guidelines

Official source data — quoted verbatim from the CMS/NCHS Official Guidelines

Verbatim excerpts from the ICD-10-CM Official Guidelines for Coding and Reporting (CMS/NCHS) that govern this code.

Chapter 9: Diseases of the Circulatory System (I00 -I99)

11) Pulmonary Hypertension Pulmonary hypertension is classified to category I27, Other pulmonary heart diseases. For secondary pulmonary hypertension (I27.1, I27.2-), code also any associated conditions or adverse effects of drugs or toxins. The sequencing is based on the reason for the encounter, except for adverse effects of drugs (See Section I.C.19.e.).

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-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name I27.841 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 Excludes2 note: Z98 — Other postprocedural states (via I27.84.-).

These codes’ tabular lists name this diagnosis as distinct — both may be reported when both are documented.

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.

Named in the grouper logic of 3 MS-DRGs: DRG 808 (MDC 16), DRG 809 (MDC 16), DRG 810 (MDC 16).

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

Clinical classification (AHRQ CCSR):CIR038 — Postprocedural or postoperative circulatory system complication (default); CIR014 — Pulmonary heart disease; CIR039 — Other specified diseases of veins and lymphatics.

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 category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical categories (Postprocedural or postoperative circulatory system complication, Pulmonary heart disease, Other specified diseases of veins and lymphatics).

I27.20 — Pulmonary hypertension, unspecified, I27.21 — Secondary pulmonary arterial hypertension, I27.22 — Pulmonary hypertension due to left heart disease, I27.23 — Pulmonary hypertension due to lung diseases and hypoxia, I27.24 — Chronic thromboembolic pulmonary hypertension, I27.29 — Other secondary pulmonary hypertension, I27.81 — Cor pulmonale (chronic), I27.82 — Chronic pulmonary embolism, I27.83 — Eisenmenger's syndrome, I27.840 — Fontan-associated liver disease [FALD], I27.848 — Other Fontan-associated condition, I27.849 — Fontan related circulation, unspecified, I27.89 — Other specified pulmonary heart diseases, I27.9 — Pulmonary heart disease, unspecified, I28.0 — Arteriovenous fistula of pulmonary vessels, I28.1 — Aneurysm of pulmonary artery, I28.8 — Other diseases of pulmonary vessels, I28.9 — Disease of pulmonary vessels, unspecified, I85.00 — Esophageal varices without bleeding, I85.01 — Esophageal varices with bleeding, +67 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Dysfunction”, “Circulation”; these codes share that main term but sit in a different category of the Tabular List.

H02.884 — Meibomian gland dysfunction left upper eyelid (meibomian gland, of eyelid, left, upper), H02.885 — Meibomian gland dysfunction left lower eyelid (meibomian gland, of eyelid, left, lower), H02.886 — Meibomian gland dysfunction of left eye, unspecified eyelid (meibomian gland, of eyelid, left), H02.889 — Meibomian gland dysfunction of unspecified eye, unspecified eyelid (meibomian gland, of eyelid), H02.88A — Meibomian gland dysfunction right eye, upper and lower eyelids (meibomian gland, of eyelid, right, upper and lower eyelids), H02.88B — Meibomian gland dysfunction left eye, upper and lower eyelids (meibomian gland, of eyelid, left, upper and lower eyelids), I20.81 — Angina pectoris with coronary microvascular dysfunction (coronary microvascular, with, angina pectoris), I21.B — Myocardial infarction with coronary microvascular dysfunction (coronary microvascular, with, myocardial infarction), I24.81 — Acute coronary microvascular dysfunction (coronary microvascular, acute), I25.85 — Chronic coronary microvascular dysfunction (coronary microvascular), I49.5 — Sick sinus syndrome (sinoatrial node), I50.9 — Heart failure, unspecified (ventricular, with congestive heart failure), I51.81 — Takotsubo syndrome (left ventricular, following sudden emotional stress), I51.89 — Other ill-defined heart diseases (heart), I51.9 — Heart disease, unspecified (ventricular), I99.8 — Other disorder of circulatory system (collateral, any site), I99.9 — Unspecified disorder of circulatory system (defective), J4A.0 — Restrictive allograft syndrome (chronic, lung allograft, mixed), J4A.8 — Other chronic lung allograft dysfunction (chronic, lung allograft, specified NEC), J4A.9 — Chronic lung allograft dysfunction, unspecified (chronic, lung allograft), +121 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Gamma-glutamyl Transferase (GGT) Test, Iron Panel, Partial Thromboplastin Time (PTT) Test, Prothrombin Time Test and INR (PT/INR)

Contextual Map

Every relationship of I27.841 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 I27.841 with these 4 related codes in Claim Check

Hierarchy

Code Also

Referenced by Excludes2 notes

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 16 — Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders[MDC crossing]: “Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders — 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. 1,615 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Circulation, Fontan related, with, Fontan-associated lymphatic dysfunction[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Dysfunction, lymphatic, Fontan-associated[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (20)

Change history

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.

Indexed Clinical Terms (2)

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.

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
Coding guidelines Official source data
ICD-10-CM Official Guidelines for Coding and Reporting (FY2027), quoted by section 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
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 — v44 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. "I27.841 — Fontan-associated lymphatic dysfunction." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/i27.841-fontan-associated-lymphatic-dysfunction

Change history

  • FY2026 — October 1, 2025
    Added to the code set
    Fontan-associated lymphatic dysfunction
    FY2026 changes

Nearest Codes in This Family

Official ICD-10-CM classifications closest to I27.841 in its code family, with their registry titles.

View all codes in the I27 family