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Q22.0 ICD-10-CM Code: Pulmonary valve atresia

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

Coding at a Glance

Risk adjustment
RxHCC V08 category 191

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.

Code Overview

Pulmonary valve atresia is a billable ICD-10-CM diagnosis code (Q22.0).

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 (7)

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

  • MCC 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 Q22.0 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 2 Excludes2 notes across 2 chapters: I08 — Multiple valve diseases (via Q22.-), P29 — Cardiovascular disorders originating in the perinatal period (via Q22.-).

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

Referenced by 1 Use Additional Code instruction: Q87.88 — CTNNB1 syndrome (via Q22.-).

These codes instruct coders to additionally report this code when it applies.

Referenced by 2 Code Also instructions across 2 chapters: I27.21 — Secondary pulmonary arterial hypertension (via Q22.-), Z71.87 — Encounter for pediatric-to-adult transition counseling (via Q22.-).

These codes suggest coding this condition alongside when both are present.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: MCC — Major 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 61 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):MAL001 — Cardiac and circulatory 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 MCC — 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.

E78.71 — Barth syndrome, E78.72 — Smith-Lemli-Opitz syndrome, P29.30 — Pulmonary hypertension of newborn, P29.38 — Other persistent fetal circulation, Q20.9 — Congenital malformation of cardiac chambers and connections, unspecified, Q22.1 — Congenital pulmonary valve stenosis, Q22.2 — Congenital pulmonary valve insufficiency, Q22.3 — Other congenital malformations of pulmonary valve, Q23.81 — Bicuspid aortic valve, Q23.82 — Congenital mitral valve cleft leaflet, Q23.88 — Other congenital malformations of aortic and mitral valves, Q23.9 — Congenital malformation of aortic and mitral valves, unspecified, Q24.8 — Other specified congenital malformations of heart, Q24.9 — Congenital malformation of heart, unspecified, Q27.30 — Arteriovenous malformation, site unspecified, Q27.4 — Congenital phlebectasia, Q28.0 — Arteriovenous malformation of precerebral vessels, Q28.1 — Other malformations of precerebral vessels, Q28.8 — Other specified congenital malformations of circulatory system, Q28.9 — Congenital malformation of circulatory system, unspecified, +40 more

Same clinical category (CCSR)

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

Q21.19 — Other specified atrial septal defect, Q21.2 — Atrioventricular septal defect, Q21.20 — Atrioventricular septal defect, unspecified as to partial or complete, Q21.21 — Partial atrioventricular septal defect, Q21.22 — Transitional atrioventricular septal defect, Q21.23 — Complete atrioventricular septal defect, Q21.3 — Tetralogy of Fallot, Q21.4 — Aortopulmonary septal defect, Q21.8 — Other congenital malformations of cardiac septa, Q21.9 — Congenital malformation of cardiac septum, unspecified, Q22.1 — Congenital pulmonary valve stenosis, Q22.2 — Congenital pulmonary valve insufficiency, Q22.3 — Other congenital malformations of pulmonary valve, Q22.4 — Congenital tricuspid stenosis, Q22.5 — Ebstein's anomaly, Q22.6 — Hypoplastic right heart syndrome, Q22.8 — Other congenital malformations of tricuspid valve, Q22.9 — Congenital malformation of tricuspid valve, unspecified, Q23.0 — Congenital stenosis of aortic valve, Q23.1 — Congenital insufficiency of aortic valve, +87 more

Same Index main term, other category

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

Q20.8 — Other congenital malformations of cardiac chambers and connections (band, atrial), Q20.9 — Congenital malformation of cardiac chambers and connections, unspecified (cardiac, chambers), Q21.0 — Ventricular septal defect (Gerbode), Q21.10 — Atrial septal defect, unspecified (atrial, septa), Q21.12 — Patent foramen ovale (foramen, ovale), Q21.19 — Other specified atrial septal defect (atrial septum), Q21.3 — Tetralogy of Fallot (heart, tetralogy of Fallot), Q21.4 — Aortopulmonary septal defect (septum, between aorta and pulmonary artery), Q21.8 — Other congenital malformations of cardiac septa (Eisenmenger's), Q21.9 — Congenital malformation of cardiac septum, unspecified (heart, septum), Q23.0 — Congenital stenosis of aortic valve (aortic), Q23.1 — Congenital insufficiency of aortic valve (heart, valve NEC, aortic, insufficiency), Q23.2 — Congenital mitral stenosis (mitral valve), Q23.3 — Congenital mitral insufficiency (mitral, insufficiency), Q23.4 — Hypoplastic left heart syndrome (aortic, in hypoplastic left heart syndrome), Q23.81 — Bicuspid aortic valve (heart, valve NEC, aortic, bicuspid valve), Q23.82 — Congenital mitral valve cleft leaflet (mitral, cleft), Q23.88 — Other congenital malformations of aortic and mitral valves (mitral, specified NEC), Q23.9 — Congenital malformation of aortic and mitral valves, unspecified (mitral), Q24.3 — Pulmonary infundibular stenosis (pulmonary, valve, stenosis, subvalvular), +425 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.

Prothrombin Time Test and INR (PT/INR)

Contextual Map

Every relationship of Q22.0 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 Q22.0 with these 5 related codes in Claim Check

Hierarchy

Referenced by Excludes2 notes

Referenced by Use Additional Code instructions

  • Q87.88 — CTNNB1 syndrome[Use Additional Code](via Q22.-): “congenital heart malformations (Q20.0-Q24.9)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Referenced by Code Also 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

  • Absence (of) (organ or part) (complete or partial), pulmonary valve[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Agenesis, heart, valve NEC, pulmonary[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Anomaly, anomalous (congenital) (unspecified type), heart, valve NEC, pulmonary, atresia[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Anomaly, anomalous (congenital) (unspecified type), pulmonary, valve, atresia[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Atresia, atretic, heart valve NEC, pulmonary[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Atresia, atretic, pulmonary (artery), valve[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Atresia, atretic, pulmonic[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (9)

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
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. "Q22.0 — Pulmonary valve atresia." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/q22.0-pulmonary-valve-atresia

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Pulmonary valve atresia

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027 (effective October 1, 2026), and none are recorded for this code. Note changes are tracked from FY2027 only.

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q22.0 in its code family, with their registry titles.

View all codes in the Q22 family