2026 ICD-10-CM Diagnosis Code E74.02Pompe disease

ICD-10-CM Codes›E00–E89›E70-E88›E74

ICD-10-CM E74.02
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E74.02 is a billable ICD-10-CM diagnosis code for pompe disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as cardiac glycogenosis. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E74.02
Billable Status
Yes — Valid for Submission
Code Describes
Pompe disease
Short Description
Pompe disease
Same as the full description in the CMS dataset.
Parent Code
E74.0
Glycogen storage disease

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE74Other disorders of carbohydrate metabolism
This CodeE74.02Pompe disease

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Cardiac glycogenosis
  • Deficiency of alpha-glucosidase
  • Deficiency of glucan 1,4-alpha-glucosidase
  • Fatal congenital nonlysosomal heart glycogenosis
  • Glycogen storage disease due to acid maltase deficiency
  • Glycogen storage disease due to acid maltase deficiency, infantile onset
  • Glycogen storage disease due to acid maltase deficiency, late-onset
  • Glycogen storage disease with severe cardiomyopathy due to glycogenin deficiency
  • Infiltrative cardiomyopathy
  • Lysosomal storage disease

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Cardiac glycogenosis
  • Type II glycogen storage disease

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • 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)

Clinical ClassificationClinical

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

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Myocardial Degeneration

    degeneration of myocardial tissue.

Patient EducationClinical

Carbohydrate Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.

Read the full article at MedlinePlus

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

Convert E74.02 to ICD-9-CMHistory

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

ICD-9-CM
271.0 Glycogenosis
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 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E74.02Overview

Is E74.02 (Glycogen storage disease) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report pompe disease on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E74.02?

Under the General Equivalence Mappings, pompe disease converts to ICD-9-CM 271.0 (glycogenosis). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.