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E74.02

Pompe disease

Billable FY2026 2025-10-01 → 2026-09-30
Exclusions 11 never code together
E74code
Excludes1
  • diabetes mellitus (E08-E13)E08-E13
  • hypoglycemia NOS (E16.2)E16.2
  • increased secretion of glucagon (E16.3)E16.3
  • mucopolysaccharidosis (E76.0-E76.3)E76.0-E76.3
E70-E88block
Excludes1
  • androgen insensitivity syndrome (E34.5-)E34.5-
  • congenital adrenal hyperplasia (E25.0)E25.0
  • hemolytic anemias attributable to enzyme disorders (D55.-)D55.-
  • Marfan syndrome (Q87.4-)Q87.4-
  • 5-alpha-reductase deficiency (E29.1)E29.1
Excludes2
  • Ehlers-Danlos syndromes (Q79.6-)Q79.6-
E00-E89chapter
Excludes1
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)P70-P74

Excludes1 — never code together. The excluded condition and this one are mutually exclusive.

Excludes2 — not included here, but the patient may have both. Code both when documented.

Instructional notes 3
E74.02code
Inclusion terms
  • Cardiac glycogenosis
  • Type II glycogen storage disease
E00-E89chapter
Notes
  • 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.E05.8E07.0E16-E31E34.-
Index entries leading here 9
  • Infiltrate, infiltration
    • cardiac
    • myocardium, myocardial
    • heart, cardiac
  • Glycogenosis (diffuse) (generalized) — see also Disease, glycogen storage
  • Cardiomyopathy (familial) (idiopathic) I42.9
    • due to
    • glycogen storage E74.02
  • Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) I42.9
    • in (due to)
    • glycogen storage E74.02
  • Cardiomegalia glycogenica diffusa E74.02
  • Cardiomegaly — see also Hypertrophy, cardiac
  • Deficiency, deficient
    • lysosomal alpha-1, 4 glucosidase E74.02
  • Disease, diseased — see also Syndrome
  • Pompe's disease (glycogen storage) E74.02
Alongside this code 6 same parent — E74.0
Additional references
AHA Coding Clinic® Licence required

Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.

Risk adjustment

CMS22HCC23 CMS28HCC49 ESRD21HCC23 ESRD24HCC23 RX08HCC41

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This code

Billable
yes
Code set
FY2026
Parent
E74.0
Block
E70-E88
Siblings
6

Present FY2024–FY2027.