2020 ICD-10-CM Diagnosis Code E74.02

Pompe disease

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E74.02 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E74.02 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E74.02 - other international versions of ICD-10 E74.02 may differ.
Applicable To
  • Cardiac glycogenosis
  • Type II glycogen storage disease
The following code(s) above E74.02 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E74.02:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    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.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E74
    ICD-10-CM Diagnosis Code E74

    Other disorders of carbohydrate metabolism

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Other disorders of carbohydrate metabolism
Approximate Synonyms
  • Generalized glycogen storage disease of infants
  • Glycogen storage disease, type 2
Clinical Information
  • An autosomal recessive inherited type of glycogen storage disease caused by deficiency of the enzyme acid alpha-glucosidase. It results in the abnormal accumulation of glycogen in the heart, skeletal muscles, liver, and nervous system.
  • 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)
  • 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, heart, liver, spinal cord, and brain; three forms have been described: the infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy; 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.
ICD-10-CM E74.02 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E74.02 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to E74.02:
  • Cardiomegalia glycogenica diffusa E74.02
  • Cardiomegaly - see also Hypertrophy, cardiac
    • glycogen E74.02
  • Cardiomyopathy (familial) (idiopathic) I42.9
    ICD-10-CM Diagnosis Code I42.9

    Cardiomyopathy, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Cardiomyopathy (primary) (secondary) NOS
    • due to
      • cardiac glycogenosis E74.02
    • glycogen storage E74.02
  • Deficiency, deficient
    • lysosomal alpha-1, 4 glucosidase E74.02
  • Disease, diseased - see also Syndrome
    • glycogen storage E74.00
      ICD-10-CM Diagnosis Code E74.00

      Glycogen storage disease, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • heart E74.02
      • myocardium E74.02
      • Pompe's E74.02
      • type II E74.02
    • heart (organic) I51.9
      ICD-10-CM Diagnosis Code I51.9

      Heart disease, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • glycogen storage E74.02
    • Pompe's E74.02 (glycogenosis II)
  • Glycogenosis (diffuse) (generalized) - see also Disease, glycogen storage
    • cardiac E74.02
  • Infiltrate, infiltration
  • Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) I42.9
    - see also Cardiomyopathy
    ICD-10-CM Diagnosis Code I42.9

    Cardiomyopathy, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Cardiomyopathy (primary) (secondary) NOS
    • in (due to)
      • cardiac glycogenosis E74.02
    • glycogen storage E74.02
  • Pompe's disease E74.02 (glycogen storage)

ICD-10-CM Codes Adjacent To E74.02
E72.9 Disorder of amino-acid metabolism, unspecified
E73 Lactose intolerance
E73.0 Congenital lactase deficiency
E73.1 Secondary lactase deficiency
E73.8 Other lactose intolerance
E73.9 Lactose intolerance, unspecified
E74 Other disorders of carbohydrate metabolism
E74.0 Glycogen storage disease
E74.00 …… unspecified
E74.01 von Gierke disease
E74.02 Pompe disease
E74.03 Cori disease
E74.04 McArdle disease
E74.09 Other glycogen storage disease
E74.1 Disorders of fructose metabolism
E74.10 Disorder of fructose metabolism, unspecified
E74.11 Essential fructosuria
E74.12 Hereditary fructose intolerance
E74.19 Other disorders of fructose metabolism
E74.2 Disorders of galactose metabolism
E74.20 …… unspecified

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.