2027 ICD-10-CM Diagnosis Code E74.02Pompe disease
ICD-10-CM Codes›E00–E89›E70-E88›E74
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Risk Adjusts — HCC 49
- Chronic Condition
E74.02 is a billable ICD-10-CM diagnosis code for pompe disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. 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 140 closely related codes. 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.
For Medicare Advantage risk adjustment, E74.02 maps to CMS-HCC Category 49 (Specified Lysosomal Storage Disorders) under the V28 model, adding a risk factor of about 9.256 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E74.02 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.
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.
- 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
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E74.02: its own notes plus those printed at E74, block E70-E88, and Chapter 4. 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.
- Cardiac glycogenosis
- Type II glycogen storage disease
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
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.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- 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.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E74.02, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E74.02: they are not reported together with it, unless the two conditions are unrelated.
- R79 Other abnormal findings of blood chemistry
- Block R80-R82 Abnormal findings on examination of urine, without diagnosis
Code First 3
These codes say to code first a condition named in the note, and E74.02 is one of the conditions named.
- G73.7 Myopathy in diseases classified elsewhereglycogen storage disease (E74.0-) names E74.0-, which includes this code
- I43 Cardiomyopathy in diseases classified elsewhereglycogen storage disease (E74.0-) names E74.0-, which includes this code
- N16 Renal tubulo-interstitial disorders in diseases classified elsewhereglycogen storage disease (E74.0-) names E74.0-, which includes this code
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Cardiomegaly See Also: Hypertrophy, cardiac;
glycogen E74.02
Disease, diseased See Also: Syndrome;
heart E74.02
lysosomal E74.02
with acid maltase deficiency E74.02
myocardium E74.02
Pompe's E74.02
type II E74.02
glycogen storage E74.02
Pompe's (glycogenosis II) E74.02
Glycogenosis (diffuse) (generalized) See Also: Disease, glycogen storage;
cardiac E74.02
glycogenic E74.02
glycogenic E74.02
glycogenic E74.02
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Glycogen Storage Disease Type II
an autosomal recessively inherited glycogen storage disease caused by glucan 1,4-alpha-glucosidase deficiency. large amounts of glycogen accumulate in the lysosomes of skeletal muscle (muscle, skeletal); heart; liver; spinal cord; and brain. three forms have been described: infantile, childhood, and adult. the infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (cardiomyopathy, hypertrophic). the childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms. the adult form consists of a slowly progressive proximal myopathy. (from muscle nerve 1995;3:s61-9; menkes, textbook of child neurology, 5th ed, pp73-4)
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.
Code HistoryHistory
Questions About E74.02Overview
What is the ICD-10 code for pompe disease?
The ICD-10-CM code for pompe disease is E74.02 (sometimes written as E7402). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
What MS-DRG does E74.02 group to?
When pompe disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Is E74.02 a CC or MCC?
CMS lists E74.02 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 140 closely related codes in its exclusion list.
Can E74.02 be reported with E16.2?
Not as a rule. The Excludes1 note at E74, which applies to E74.02, lists "hypoglycemia NOS (E16.2)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What HCC is E74.02?
E74.02 (pompe disease) maps to CMS-HCC Category 49 (Specified Lysosomal Storage Disorders), commonly written as HCC 49, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 23 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 41.
Does E74.02 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, E74.02 adds a risk adjustment factor of about 9.256 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 1.528 to 13.778 depending on the payment segment). HCC 49 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 49 category page.
