E74.02
Pompe disease
E74.02 (also written E7402) is a billable ICD-10-CM diagnosis code for pompe disease. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 24 codes in category E74 (Other disorders of carbohydrate metabolism). The Alphabetic Index lists it under 20 entries. Its coding notes include 10 Excludes1 entries and 1 Excludes2 entry.
Excludes1
Not coded here. The listed conditions generally should not be reported together.
Also from chapter/block level (6 notes)
Excludes2
Not included here. Both codes may be reported if the patient has both conditions.
Care settings
How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.
Home Health Primary
Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the MMTA - Other clinical group.
Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026Hospital CC
Counts as a complication or comorbidity (CC) when reported as a secondary diagnosis on an inpatient claim. It does not count when the principal diagnosis is one of the 140 codes in its exclusion list. Some MS-DRGs also ignore a CC or MCC that defines the DRG.
Source: CMS MS-DRG Definitions Manual v44, effective October 1, 2026, Appendix CInclusion terms
Conditions classified to this code.
- Cardiac glycogenosis
- Type II glycogen storage disease
Notes
Also from chapter/block level (1 note)
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Alphabetic Index terms
Terms the official Alphabetic Index lists for this code. 20 entries
- 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
Show 8 more
- 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)