Q25.47 ICD-10-CM Code: Right aortic arch

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q25.47 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 Q25.47 itself; “inherited from” names the category or block whose note applies here.

Coder workflow for Q25.47

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

Before you code Q25.47

1. Laterality is coded in this family. Confirm the side documented — right, left, or bilateral — and select the matching code (this page’s code: right). The unspecified-side code applies only when the record states no side. Laterality is assigned from the documented side; where a bilateral code exists and both sides are documented, it is used instead of two unilateral codes (Guidelines I.B.13).

   See the relationships section · Guide: Laterality coding →

2. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with Q25.47. 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 Q25.47’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.

   ReviewQ23.4

2. Is the side documented?
   Yes → Select the code for the documented side (or the bilateral code when both sides are documented and one exists).
   No → Use the unspecified-side code only when the record states no side; a query is the alternative.

Consider Q25.47. 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).

Laterality

Right, left or bilateral as documented; unspecified only when the record states no side (Guidelines I.B.13).

Official instructions as workflow

• Excludes1 — check before selecting Q25.47(1 note)

  Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with Q25.47: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

  CompareQ23.4

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

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 (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.

• Persistence, persistent (congenital), right aortic arch
• Right aortic arch

Don’t confuse Q25.47 with…

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

The codes most likely to be picked instead of Q25.47 — 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.

• Q23.4— 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 hypoplasia of aorta in hypoplastic left heart syndrome moves the case to Q23.4; the two are not reported together unless documented as unrelated.

  Full comparison: Q25.47 vs Q23.4 →

• Q25.40— 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 “Congenital malformation of aorta unspecified” takes Q25.40; specificity in the record moves it back here.

  Full comparison: Q25.47 vs Q25.40 →

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.

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 Q25.47 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: P29 — Cardiovascular disorders originating in the perinatal period (via Q25.-).

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

Referenced by 2 Code Also instructions across 2 chapters: I27.21 — Secondary pulmonary arterial hypertension (via Q25.-), Z71.87 — Encounter for pediatric-to-adult transition counseling (via Q25.-).

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: 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 56 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 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.

Q25.21 — Interruption of aortic arch, Q25.29 — Other atresia of aorta, Q25.3 — Supravalvular aortic stenosis, Q25.40 — Congenital malformation of aorta unspecified, Q25.41 — Absence and aplasia of aorta, Q25.42 — Hypoplasia of aorta, Q25.43 — Congenital aneurysm of aorta, Q25.44 — Congenital dilation of aorta, Q25.45 — Double aortic arch, Q25.46 — Tortuous aortic arch, Q25.48 — Anomalous origin of subclavian artery, Q25.49 — Other congenital malformations of aorta, Q25.8 — Other congenital malformations of other great arteries, Q25.9 — Congenital malformation of great arteries, 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, +35 more

Same clinical category (CCSR)

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

Q25.29 — Other atresia of aorta, Q25.3 — Supravalvular aortic stenosis, Q25.4 — Other congenital malformations of aorta, Q25.40 — Congenital malformation of aorta unspecified, Q25.41 — Absence and aplasia of aorta, Q25.42 — Hypoplasia of aorta, Q25.43 — Congenital aneurysm of aorta, Q25.44 — Congenital dilation of aorta, Q25.45 — Double aortic arch, Q25.46 — Tortuous aortic arch, Q25.48 — Anomalous origin of subclavian artery, Q25.49 — Other congenital malformations of aorta, Q25.5 — Atresia of pulmonary artery, Q25.6 — Stenosis of pulmonary artery, Q25.71 — Coarctation of pulmonary artery, Q25.72 — Congenital pulmonary arteriovenous malformation, Q25.79 — Other congenital malformations of pulmonary artery, Q25.8 — Other congenital malformations of other great arteries, Q25.9 — Congenital malformation of great arteries, unspecified, Q26.0 — Congenital stenosis of vena cava, +87 more

Same Index main term, other category

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

Q14.8 — Other congenital malformations of posterior segment of eye (hyaloid, system), Q16.3 — Congenital malformation of ear ossicles (arteria stapedia), Q18.0 — Sinus, fistula and cyst of branchial cleft (branchial cleft NOS, cyst), Q18.2 — Other branchial cleft malformations (branchial cleft NOS), Q20.0 — Common arterial trunk (truncus arteriosus or communis), Q21.11 — Secundum atrial septal defect (ostium, secundum), Q21.12 — Patent foramen ovale (foramen, ovale), Q21.20 — Atrioventricular septal defect, unspecified as to partial or complete (ostium, primum), Q21.23 — Complete atrioventricular septal defect (ostium, atrioventriculare commune), Q21.8 — Other congenital malformations of cardiac septa (bulbus cordis in left ventricle), Q26.1 — Persistent left superior vena cava (left, superior vena cava), Q26.8 — Other congenital malformations of great veins (left, posterior cardinal vein), Q42.2 — Congenital absence, atresia and stenosis of anus with fistula (anal membrane, with fistula), Q42.3 — Congenital absence, atresia and stenosis of anus without fistula (anal membrane), Q43.0 — Meckel's diverticulum (displaced) (hypertrophic) (vitelline duct), Q43.7 — Persistent cloaca (cloaca), Q43.8 — Other specified congenital malformations of intestine (pancreatic tissue in intestinal tract), Q50.6 — Other congenital malformations of fallopian tube and broad ligament (convolutions, oviduct), Q51.828 — Other congenital malformations of cervix (fetal, form of cervix), Q52.4 — Other congenital malformations of vagina (Gartner's duct), +14 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.

Lipid Panel

Contextual Map

Every relationship of Q25.47 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 Q25.47 with these 3 related codes in Claim Check

Hierarchy

• Q00-QA1 — Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-QA1) (Q00-QA1)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026

Referenced by Excludes2 notes

• P29 — Cardiovascular disorders originating in the perinatal period[Excludes2](via Q25.-): “congenital malformations of the circulatory system (Q20-Q28)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Referenced by Code Also instructions

• I27.21 — Secondary pulmonary arterial hypertension[Code Also](via Q25.-): “congenital heart disease (Q20-Q28)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
• Z71.87 — Encounter for pediatric-to-adult transition counseling[Code Also](via Q25.-): “congenital malformations of the circulatory system (Q20-Q28)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Clinical classification (CCSR)

• MAL001 — Cardiac and circulatory congenital anomalies[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)

MS-DRG Grouper

• CC — Complication or Comorbidity [MS-DRG severity]: “As a secondary diagnosis this code can raise the stay's MS-DRG severity tier (CC).”— CMS MS-DRG Definitions Manual (Appendix C) · FY2026
• DRG 306 — CARDIAC CONGENITAL AND VALVULAR DISORDERS WITH MCC[MS-DRG]: “CARDIAC CONGENITAL AND VALVULAR DISORDERS WITH MCC (MDC 05)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 307 — CARDIAC CONGENITAL AND VALVULAR DISORDERS WITHOUT MCC[MS-DRG]: “CARDIAC CONGENITAL AND VALVULAR DISORDERS WITHOUT MCC (MDC 05)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026

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

• Persistence, persistent (congenital), right aortic arch[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
• Right aortic arch[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (25)

• Q25 — Congenital malformations of great arteries[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.0 — Patent ductus arteriosus[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.1 — Coarctation of aorta[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.2 — Atresia of aorta[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.21 — Interruption of aortic arch[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.29 — Other atresia of aorta[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.3 — Supravalvular aortic stenosis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q25.4 — Other congenital malformations of aorta[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• and 17 more

Change history

• FY2017 — Added to the code set[Change history]— CMS release files (code change ledger) · icd10cm-fy2017

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

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q25.47 in its code family, with their registry titles.

• Q25.42 — Hypoplasia of aorta
• Q25.43 — Congenital aneurysm of aorta
• Q25.44 — Congenital dilation of aorta
• Q25.45 — Double aortic arch
• Q25.46 — Tortuous aortic arch
• Q25.48 — Anomalous origin of subclavian artery
• Q25.49 — Other congenital malformations of aorta
• Q25.5 — Atresia of pulmonary artery
• Q25.6 — Stenosis of pulmonary artery
• Q25.7 — Other congenital malformations of pulmonary artery

View all codes in the Q25 family