2024 ICD-10-CM Diagnosis Code G71.3

Mitochondrial myopathy, not elsewhere classified

ICD-10-CM Code:
ICD-10 Code for:
Mitochondrial myopathy, not elsewhere classified
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Diseases of the nervous system
    • Diseases of myoneural junction and muscle
      • Primary disorders of muscles

G71.3 is a billable diagnosis code used to specify a medical diagnosis of mitochondrial myopathy, not elsewhere classified. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy
  • Adult-onset multiple mitochondrial deoxyribonucleic acid deletion syndrome due to deoxyguanosine kinase deficiency
  • Autosomal dominant mitochondrial myopathy with exercise intolerance
  • Childhood myocerebrohepatopathy spectrum
  • Congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy syndrome
  • Cytochrome-c oxidase deficiency
  • DNA2-related mitochondrial DNA deletion syndrome
  • FASTKD2-related infantile mitochondrial encephalomyopathy
  • FBXL4-related encephalomyopathic mitochondrial DNA depletion syndrome
  • Hereditary cerebellar atrophy
  • Hypertrophic mitochondrial cardiomyopathy
  • Lethal infantile mitochondrial myopathy
  • Maternally inherited mitochondrial cardiomyopathy
  • Maternally inherited mitochondrial cardiomyopathy and myopathy
  • Maternally inherited mitochondrial deoxyribonucleic acid disease
  • Maternally inherited mitochondrial deoxyribonucleic acid disease
  • Maternally inherited mitochondrial deoxyribonucleic acid disease
  • Maternally inherited mitochondrial myopathy
  • Mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form with methylmalonic aciduria
  • Mitochondrial DNA depletion syndrome, encephalomyopathic form with variable craniofacial anomalies
  • Mitochondrial encephalomyopathy
  • Mitochondrial myopathy
  • Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
  • Mitochondrial myopathy with sideroblastic anemia syndrome
  • Mitochondrial myopathy, cerebellar ataxia, pigmentary retinopathy syndrome
  • Mitochondrial myopathy, lactic acidosis, deafness syndrome
  • Mitochondrial neurogastrointestinal encephalomyopathy syndrome
  • Myopathy and diabetes mellitus
  • Progressive external ophthalmoplegia, myopathy, emaciation syndrome
  • Pure mitochondrial myopathy
  • RRM2B-related mitochondrial DNA depletion syndrome, encephalomyopathic form with renal tubulopathy
  • Severe X-linked mitochondrial encephalomyopathy
  • TK2-related mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
  • TMEM70 related mitochondrial encephalo-cardio-myopathy

Clinical Classification

Clinical Information

  • Mitochondrial Myopathy

    myopathy caused by mitochondrial abnormalities.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • Kearns-Sayre syndrome H49.81
  • Leber's disease H47.21
  • Leigh's encephalopathy G31.82
  • mitochondrial metabolism disorders E88.4
  • Reye's syndrome G93.7

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert G71.3 to ICD-9-CM

  • ICD-9-CM Code: 359.89 - Myopathies NEC
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Mitochondrial Diseases

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. If you have a metabolic disorder, something goes wrong with this process.

Mitochondrial diseases are a group of metabolic disorders. Mitochondria are small structures that produce energy in almost all of your cells. They make it by combining oxygen with the fuel molecules (sugars and fats) that come from your food. When the mitochondria are defective, the cells do not have enough energy. The unused oxygen and fuel molecules build up in the cells and cause damage.

The symptoms of mitochondrial disease can vary. It depends on how many mitochondria are defective, and where they are in the body. Sometimes only one organ, tissue, or cell type is affected. But often the problem affects many of them. Muscle and nerve cells have especially high energy needs, so muscular and neurological problems are common. The diseases range from mild to severe. Some types can be fatal.

Genetic mutations cause these diseases. They usually happen before age 20, and some are more common in infants. There are no cures for these diseases, but treatments may help with symptoms and slow down the disease. They may include physical therapy, vitamins and supplements, special diets, and medicines.

[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.


[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.