2027 ICD-10-CM Diagnosis Code Q44.71Alagille syndrome

ICD-10-CM Codes›Q00-Q99›Q38-Q45›Q44

ICD-10-CM Q44.71
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q44.71 is a billable ICD-10-CM diagnosis code for alagille syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 441 through 443. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 10 closely related codes. The code is exempt from POA reporting. Coders also document this condition as arteriohepatic dysplasia. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Digestive congenital anomalies.

For Medicare Advantage risk adjustment, Q44.71 maps to CMS-HCC Category 68 (Cholangitis and Obstruction of Bile Duct Without Gallstones) under the V28 model, adding a risk factor of about 0.388 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
Q44.71
Billable Status
Yes — Valid for Submission
Code Describes
Alagille syndrome
Short Description
Alagille syndrome
Same as the full description in the CMS dataset.
Parent Code
Q44.7
Other congenital malformations of liver

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ38-Q45Other congenital malformations of the digestive system
CategoryQ44Congenital malformations of gallbladder, bile ducts and liver
This CodeQ44.71Alagille syndrome

Present on Admission (POA)Billing

Q44.71 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations of the digestive system (Q38-Q45).

Medicare Risk Adjustment (HCC)Billing

Q44.71 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 68— Cholangitis and Obstruction of Bile Duct Without Gallstones
Payment HCC · PY 2027 one of 8 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+0.388
community, non-dual, aged · ranges 0.085–0.391 across segments
Hierarchy
Superseded by HCC 62, HCC 63, and HCC 64
a more severe related category takes the payment when both are reported
Prior Model (CMS-HCC V24)
Not mapped under V24
this diagnosis newly risk-adjusts under the V28 model
Part D (RxHCC)
Not mapped
Q44.71 does not risk-adjust in the RxHCC prescription drug model

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Arteriohepatic dysplasia
  • Biliary tract dysplasia

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q44.71: its own notes plus those printed at Q44.7 and Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • Alagille-Watson syndrome

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)

Code Also

A second code may be needed to fully describe the condition; the note gives no sequencing direction.

From Q44.7 Other congenital malformations of liver applies to 3 codes
  • if applicable, associated malformations affecting other systems

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 MAL002
Digestive congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Alagille Syndrome

    a multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (bile ducts, intrahepatic), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. major clinical features include jaundice, and congenital heart disease with peripheral pulmonary stenosis. alagille syndrome may result from heterogeneous gene mutations, including mutations in jag1 on chromosome 20 (type 1) and notch2 on chromosome 1 (type 2).

Code History & ChangesHistory

Replacement Q44.71 replaces the following previously assigned code(s):

  • Q44.7 - Other congenital malformations of liver
FY 2024AddedAdded to the ICD-10-CM code setEffective October 1, 2023.
FY 2025–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q44.71Overview

What is the ICD-10 code for alagille syndrome?

The ICD-10-CM code for alagille syndrome is Q44.71 (sometimes written as Q4471). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q44.71 (Other congenital malformations of liver) a billable code?

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

What MS-DRG does Q44.71 group to?

When alagille syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 442, 441, 443, with relative weights from 0.6890 to 1.8226 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q44.71 a CC or MCC?

CMS lists Q44.71 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 10 closely related codes in its exclusion list.

Is Q44.71 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 alagille syndrome on inpatient claims. The code appears in the Other congenital malformations of the digestive system (Q38-Q45) range of the CMS exempt list.

What HCC is Q44.71?

Q44.71 (alagille syndrome) maps to CMS-HCC Category 68 (Cholangitis and Obstruction of Bile Duct Without Gallstones), commonly written as HCC 68, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It does not map to any RxHCC in the Part D prescription drug model.

Does Q44.71 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, Q44.71 adds a risk adjustment factor of about 0.388 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.085 to 0.391 depending on the payment segment). A more severe related category (HCC 62, HCC 63, and HCC 64) supersedes it when both are reported. See the full factor table on the HCC 68 category page.