K76.81 ICD-10-CM Code: Hepatopulmonary syndrome

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for K76.81 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 K76.81 itself; “inherited from” names the category or block whose note applies here.

Coder workflow for K76.81

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

Before you code K76.81

1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with K76.81. 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 K76.81’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.

   ReviewR17

2. Is the underlying (etiologic) condition the Code First note names documented?
   Yes → Sequence the underlying condition first, then K76.81.
   No → Continue; do not add an underlying condition the record does not document.

   ReviewK70.3, K74.6

Consider K76.81. Then 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 underlying (etiologic) condition

Named in the Code First note; sequenced before this code when documented (Guidelines I.A.13).

Official instructions as workflow

• Excludes1 — check before selecting K76.81(1 note)

  Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with K76.81: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

  CompareR17

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

• Excludes2 — not part of K76.81(12 notes)

  Coding workflow: The conditions named in this note are not included in K76.81. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

  CompareK70, E85, Q44.6, I82.0, R16.0, E83.110

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

• Code First — sequencing check(3 notes)

  Coding workflow: Check whether the underlying or etiologic condition the note names is documented. When it is, sequence it before K76.81. Do not add an underlying condition the record does not document.

  ReviewK70.3, K74.6

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

Coding decision scenarios

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

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.

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.

• Syndrome, hepatopulmonary

Official Coding Guidelines

No excerpt in the ICD-10-CM Official Guidelines names this code specifically. Its chapter carries only this chapter-wide note:

Don’t confuse K76.81 with…

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

The codes most likely to be picked instead of K76.81 — 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.

• R17— Excludes1 conflict (Excludes1 — generally not reported together)

  Not coded here — the tabular list excludes this pair for the same encounter.

  Not reported together with this code, except where the two conditions are documented as unrelated.

  What moves you there: Documentation of jaundice NOS moves the case to R17; the two are not reported together unless documented as unrelated.

  Full comparison: K76.81 vs R17 →

• K70— Excludes2 relationship (Informational)

  Distinct conditions — both may be reported.

  A distinct condition — both may be reported when both are documented.

  What moves you there: Documentation of alcoholic liver disease adds K70; it is not part of this code and both may be reported when both are documented.

  Full comparison: K76.81 vs K70 →

• E85— Excludes2 relationship (Informational)

  Distinct conditions — both may be reported.

  A distinct condition — both may be reported when both are documented.

  What moves you there: Documentation of amyloid degeneration of liver adds E85; it is not part of this code and both may be reported when both are documented.

  Full comparison: K76.81 vs E85 →

• Q44.6— Excludes2 relationship (Informational)

  Distinct conditions — both may be reported.

  A distinct condition — both may be reported when both are documented.

  What moves you there: Documentation of cystic disease of liver (congenital) adds Q44.6; it is not part of this code and both may be reported when both are documented.

  Full comparison: K76.81 vs Q44.6 →

• I82.0— Excludes2 relationship (Informational)

  Distinct conditions — both may be reported.

  A distinct condition — both may be reported when both are documented.

  What moves you there: Documentation of hepatic vein thrombosis adds I82.0; it is not part of this code and both may be reported when both are documented.

  Full comparison: K76.81 vs I82.0 →

• R16.0— Excludes2 relationship (Informational)

  Distinct conditions — both may be reported.

  A distinct condition — both may be reported when both are documented.

  What moves you there: Documentation of hepatomegaly NOS adds R16.0; it is not part of this code and both may be reported when both are documented.

  Full comparison: K76.81 vs R16.0 →

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.

Decision Points

The directives on this code's own record, as a pre-claim checklist.

1. Sequencing: 3 Code First instructions — the underlying condition is sequenced before this code when present. See the Code First notes
2. 1 Excludes1 entry — codes named there are generally not reported together with this code (Guidelines I.A.12.a). See the Excludes1 notes
3. 12 Excludes2 entries — those conditions are not part of this code and may be reported additionally when documented. See the Excludes2 notes

Checklist rows are derived from this code's own official directives; the wording of each check is MedCoder editorial. The official notes themselves are in the sections each row links to.

Verify Before Coding

• Principal-diagnosis restriction. The Medicare Code Editor lists this code as unacceptable as a principal diagnosis: it describes a circumstance influencing health status rather than a current illness or injury being treated. It is valid as a secondary diagnosis.

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

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 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 2 MS-DRGs: DRG 205 (MDC 04), DRG 206 (MDC 04).

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

Clinical classification (AHRQ CCSR):DIG019 — Other specified and unspecified liver disease (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

Principal diagnosis restriction (Medicare Code Editor)

Not acceptable as a principal diagnosis on an inpatient claim.

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Chronic Liver Failure/End-Stage Liver Disorders) for risk-adjusted payment.

I85.00 — Esophageal varices without bleeding, I85.01 — Esophageal varices with bleeding, I85.10 — Secondary esophageal varices without bleeding, I85.11 — Secondary esophageal varices with bleeding, K70.40 — Alcoholic hepatic failure without coma, K70.41 — Alcoholic hepatic failure with coma, K72.10 — Chronic hepatic failure without coma, K72.11 — Chronic hepatic failure with coma, K72.90 — Hepatic failure, unspecified without coma, K72.91 — Hepatic failure, unspecified with coma, K76.6 — Portal hypertension, K76.7 — Hepatorenal syndrome, K76.82 — Hepatic encephalopathy

Related risk categories

These categories interact through CMS's HCC hierarchy — one can suppress the other's risk-adjustment weight when both are present on a claim.

Liver Transplant Status/Complications, Coma, Brain Compression/Anoxic Damage, Cirrhosis of Liver, Chronic Hepatitis, Cholangitis and Obstruction of Bile Duct Without Gallstones

Same clinical category (CCSR)

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

K75.89 — Other specified inflammatory liver diseases, K75.9 — Inflammatory liver disease, unspecified, K76.0 — Fatty (change of) liver, not elsewhere classified, K76.1 — Chronic passive congestion of liver, K76.2 — Central hemorrhagic necrosis of liver, K76.3 — Infarction of liver, K76.4 — Peliosis hepatis, K76.5 — Hepatic veno-occlusive disease, K76.6 — Portal hypertension, K76.7 — Hepatorenal syndrome, K76.82 — Hepatic encephalopathy, K76.89 — Other specified diseases of liver, K76.9 — Liver disease, unspecified, K77 — Liver disorders in diseases classified elsewhere, K91.83 — Postprocedural hepatorenal syndrome, O26.611 — Liver and biliary tract disorders in pregnancy, first trimester, O26.612 — Liver and biliary tract disorders in pregnancy, second trimester, O26.613 — Liver and biliary tract disorders in pregnancy, third trimester, O26.619 — Liver and biliary tract disorders in pregnancy, unspecified trimester, O26.62 — Liver and biliary tract disorders in childbirth, +33 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.

Alpha-Fetoprotein (AFP) Test, Gamma-glutamyl Transferase (GGT) Test, Hepatitis Testing, Iron Panel, Lipid Panel, Partial Thromboplastin Time (PTT) Test, Prothrombin Time Test and INR (PT/INR)

Contextual Map

Every relationship of K76.81 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 K76.81 with these 2 related codes in Claim Check

Hierarchy

• K00-K95 — Chapter 11: Diseases of the Digestive System (K00-K95) (K00-K95)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K70-K77 — Diseases of liver[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026

Code First

• K70.3 — Alcoholic cirrhosis of liver[Code First]: “alcoholic cirrhosis of liver (K70.3-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
• K74.6 — Other and unspecified cirrhosis of liver[Code First]: “cirrhosis of liver without mention of alcohol (K74.6-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Clinical classification (CCSR)

• DIG019 — Other specified and unspecified liver disease[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)

Risk adjustment (CMS-HCC)

• HCC 63 — Chronic Liver Failure/End-Stage Liver Disorders [CMS-HCC]: “Chronic Liver Failure/End-Stage Liver Disorders — supersedes HCC 64 (Cirrhosis of Liver), HCC 65 (Chronic Hepatitis), HCC 68 (Cholangitis and Obstruction of Bile Duct Without Gallstones), HCC 202 (Coma, Brain Compression/Anoxic Damage)”— CMS-HCC V28 · 2026

MS-DRG Grouper

• DRG 205 — OTHER RESPIRATORY SYSTEM DIAGNOSES WITH MCC[MS-DRG]: “OTHER RESPIRATORY SYSTEM DIAGNOSES WITH MCC (MDC 04)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 206 — OTHER RESPIRATORY SYSTEM DIAGNOSES WITHOUT MCC[MS-DRG]: “OTHER RESPIRATORY SYSTEM DIAGNOSES WITHOUT MCC (MDC 04)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026

MDC crossing

• MDC 04 — Diseases and Disorders of the Respiratory System[MDC crossing]: “Diseases and Disorders of the Respiratory 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. 3,998 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries

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

Nearest codes (14)

• K76 — Other diseases of liver[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.0 — Fatty (change of) liver, not elsewhere classified[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.1 — Chronic passive congestion of liver[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.2 — Central hemorrhagic necrosis of liver[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.3 — Infarction of liver[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.4 — Peliosis hepatis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.5 — Hepatic veno-occlusive disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• K76.6 — Portal hypertension[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• and 6 more

Change history

• FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

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

Coding guidelines Official source data

ICD-10-CM Official Guidelines for Coding and Reporting (FY2026), quoted by section 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

Risk adjustment Official source data

2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release 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

Nearest Codes in This Family

Official ICD-10-CM classifications closest to K76.81 in its code family, with their registry titles.

• K76.3 — Infarction of liver
• K76.4 — Peliosis hepatis
• K76.5 — Hepatic veno-occlusive disease
• K76.6 — Portal hypertension
• K76.7 — Hepatorenal syndrome
• K76.8 — Other specified diseases of liver
• K76.82 — Hepatic encephalopathy
• K76.83 — Intestinal failure-associated liver disease
• K76.89 — Other specified diseases of liver
• K76.9 — Liver disease, unspecified

View all codes in the K76 family