2027 ICD-10-CM Diagnosis Code Q26.5Anomalous portal venous connection

ICD-10-CM Codes›Q00-Q99›Q20-Q28›Q26

ICD-10-CM Q26.5
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q26.5 is a billable ICD-10-CM diagnosis code for anomalous portal venous connection. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 299 through 301. The code is exempt from POA reporting. Coders also document this condition as anomalous portal vein termination. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Cardiac and circulatory congenital anomalies.

Code Identity

ICD-10-CM Code
Q26.5
Billable Status
Yes — Valid for Submission
Code Describes
Anomalous portal venous connection
Short Description
Anomalous portal venous connection
Same as the full description in the CMS dataset.
Parent Code
Q26
Congenital malformations of great veins

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ20-Q28Congenital malformations of the circulatory system
CategoryQ26Congenital malformations of great veins
This CodeQ26.5Anomalous portal venous connection

Present on Admission (POA)Billing

Q26.5 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Congenital malformations of the circulatory system (Q20-Q28).

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Anomalous portal vein termination
  • Congenital absence of portal vein
  • Congenital extrahepatic portal-systemic shunt
  • Congenital intrahepatic portal-systemic shunt
  • Congenital partial portal-systemic shunt
  • Congenital portal-systemic shunt
  • Congenital splenorenal shunt
  • Partial portal-systemic vascular shunt
  • Portal systemic vascular shunt disorder

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q26.5: its own notes plus those printed at Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL001
Cardiac and circulatory congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Congenital Heart Defects

Congenital heart defects (CHDs) are problems with the structure of the heart. "Congenital" means that that the problems are present at birth. These defects happen when a fetus's heart doesn't develop normally during pregnancy. Congenital heart defects are the most common type of birth defect.

The full article covers:

  • What are congenital heart defects?
  • What causes congenital heart defects?
  • Who is more likely to have a baby with a congenital heart defect?
  • What are the symptoms of congenital heart defects?
  • What other problems do congenital heart defects cause?
  • How are congenital heart defects diagnosed?
  • What are the treatments for congenital heart defects?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q26.5 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
747.61 Gstrontest vesl anomaly
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q26.5Overview

What is the ICD-10 code for anomalous portal venous connection?

The ICD-10-CM code for anomalous portal venous connection is Q26.5 (sometimes written as Q265). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q26.5 (Congenital malformations of great veins) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report anomalous portal venous connection on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does Q26.5 group to?

When anomalous portal venous connection is the principal diagnosis on an inpatient stay, it groups to MS-DRG 301, 300, 299, with relative weights from 0.7132 to 1.5840 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q26.5 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for anomalous portal venous connection on inpatient claims. The code appears in the Congenital malformations of the circulatory system (Q20-Q28) range of the CMS exempt list.

What is the ICD-9 equivalent of Q26.5?

Under the General Equivalence Mappings, anomalous portal venous connection converts to ICD-9-CM 747.61 (gstrontest vesl anomaly). The mapping is approximate, so confirm the match fits the documentation.