ICD-10-CM 2027 diagnosis code
E74.02Pompe disease
E74.02 is a valid, billable ICD-10-CM code for pompe disease. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.
Looking up by condition? See ICD-10 code for Cardiomegalia glycogenica diffusa.
Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data
About E74.02
E74.02 is the ICD-10-CM diagnosis code for Pompe disease. It belongs to category E74 (other disorders of carbohydrate metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.
Within E74.0 (glycogen storage disease), E74.02 is specifically for Pompe disease. Related codes cover unspecified (E74.00), von Gierke disease (E74.01), Cori disease (E74.03), McArdle disease (E74.04), Lysosome-associated membrane protein 2 [LAMP2] deficiency (E74.05) and other glycogen storage disease (E74.09).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.
Before ICD-10, this condition was coded in ICD-9-CM as 271.0 (glycogenosis).
Coding notes
- Cardiac glycogenosis
- Type II glycogen storage disease
Notes that apply from higher levels
Instructions written at a parent level also apply to E74.02.
Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.
Alphabetic index entries
20 entriesTerms in the official ICD-10-CM index that lead to E74.02.
- Cardiomegalia glycogenica diffusa
- Cardiomegaly › glycogen
- Cardiomyopathy (familial) (idiopathic) › due to › cardiac glycogenosis
- Cardiomyopathy (familial) (idiopathic) › glycogen storage
- Deficiency, deficient › lysosomal alpha-1, 4 glucosidase
- Disease, diseased › glycogen storage › heart
- Disease, diseased › glycogen storage › lysosomal
- Disease, diseased › glycogen storage › lysosomal › with acid maltase deficiency
- Disease, diseased › glycogen storage › myocardium
- Disease, diseased › glycogen storage › Pompe's
- Disease, diseased › glycogen storage › type II
- Disease, diseased › heart (organic) › glycogen storage
- Disease, diseased › Pompe's (glycogenosis II)
- Glycogenosis (diffuse) (generalized) › cardiac
- Infiltrate, infiltration › cardiac › glycogenic
- Infiltrate, infiltration › heart, cardiac › glycogenic
- Infiltrate, infiltration › myocardium, myocardial › glycogenic
- Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) › glycogen storage
- Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) › in (due to) › cardiac glycogenosis
- Pompe's disease (glycogen storage)
MS-DRG v44.0 grouping
All DRGs →Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.
MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders
ICD-9-CM equivalent
Converter →- 271.0Glycogenosisapproximate
From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.
Code history
- 2016
- 2017
- 2018
- 2019
- 2020
- 2021
- 2022
- 2023
- 2024
- 2025
- 2026
- 2027
- FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
- No changes since FY2016.
Common questions about E74.02
Is E74.02 billable?
- Yes. E74.02 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E74.02 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E74.02 as a principal diagnosis.
Is E74.02 a CC or MCC?
- E74.02 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.
What DRG does E74.02 group to?
- E74.02 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.
What is the ICD-9 code for E74.02?
- The CMS General Equivalence Mappings map E74.02 to ICD-9-CM 271.0.