2022 ICD-10-CM Code G71.3

Mitochondrial myopathy, not elsewhere classified

Version 2021

Valid for Submission

ICD-10:G71.3
Short Description:Mitochondrial myopathy, not elsewhere classified
Long Description:Mitochondrial myopathy, not elsewhere classified

Code Classification

  • Diseases of the nervous system (G00–G99)
    • Diseases of myoneural junction and muscle (G70-G73)
      • Primary disorders of muscles (G71)

G71.3 is a billable diagnosis code used to specify a medical diagnosis of mitochondrial myopathy, not elsewhere classified. The code G71.3 is valid during the fiscal year 2022 from October 01, 2021 through September 30, 2022 for the submission of HIPAA-covered transactions.

The ICD-10-CM code G71.3 might also be used to specify conditions or terms like adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy, adult-onset multiple mitochondrial deoxyribonucleic acid deletion syndrome due to deoxyguanosine kinase deficiency, childhood myocerebrohepatopathy spectrum, congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy syndrome, cytochrome-c oxidase deficiency , dna2-related mitochondrial dna deletion syndrome, etc.

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code G71.3:


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.

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code G71.3 are found in the index:

Approximate Synonyms

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

Convert G71.3 to ICD-9 Code

The General Equivalency Mapping (GEM) crosswalk indicates an approximate mapping between the ICD-10 code G71.3 its ICD-9 equivalent. The approximate mapping means there is not an exact match between the ICD-10 code and the ICD-9 code and the mapped code is not a precise representation of the original code.

Information for Patients


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 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 (First year ICD-10-CM implemented into the HIPAA code set)