2027 ICD-10-CM Diagnosis Code G71.3Mitochondrial myopathy, not elsewhere classified
ICD-10-CM Codes›G00–G99›G70-G73›G71
- Billable — Valid for Submission
- Chronic Condition
G71.3 is a billable ICD-10-CM diagnosis code for mitochondrial myopathy, not elsewhere classified. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 91 through 93. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Myopathies.
G71.3 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model. It still risk-adjusts in the ESRD (V21) category 75 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
G71.3 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model, but it maps to payment categories in the other CMS models shown below.
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.
- 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
- Combined oxidative phosphorylation defect type 39
- 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
- Hypertrophic mitochondrial cardiomyopathy
- Lethal infantile mitochondrial myopathy
- Maternally inherited mitochondrial cardiomyopathy
- Maternally inherited mitochondrial cardiomyopathy and myopathy
- Maternally inherited mitochondrial myopathy
- Mitochondrial deoxyribonucleic acid depletion syndrome myopathic 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
- SLC25A42 gene-related mitochondrial encephalomyopathy
- TK2-related mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
- TMEM70 related mitochondrial encephalo-cardio-myopathy
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to G71.3: its own notes plus those printed at G71 and Chapter 6. A note printed at a category, block or chapter applies to every code under it.
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.
- certain conditions originating in the perinatal period (P04-P96)
- certain infectious and parasitic diseases (A00-B99)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name G71.3, its category, or a range that includes it.
Excludes1 1
These codes carry an Excludes1 note naming G71.3: they are not reported together with it, unless the two conditions are unrelated.
- E88.4 Mitochondrial metabolism disordersMitochondrial myopathy, NEC (G71.3)
Excludes2 1
These codes carry an Excludes2 note naming G71.3: its condition is not part of those codes, and both may be reported when the patient has both.
- Block M60-M63 Disorders of muscles
Code First 1
These codes say to code first a condition named in the note, and G71.3 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
mitochondrial NEC G71.3
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Mitochondrial Myopathies
a group of muscle diseases associated with abnormal mitochondria function.
Patient EducationClinical
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.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert G71.3 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About G71.3Overview
What is the ICD-10 code for mitochondrial myopathy, not elsewhere classified?
The ICD-10-CM code for mitochondrial myopathy, not elsewhere classified is G71.3 (sometimes written as G713). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is G71.3 (Primary disorders of muscles) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report mitochondrial myopathy, not elsewhere classified on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does G71.3 group to?
When mitochondrial myopathy, not elsewhere classified is the principal diagnosis on an inpatient stay, it groups to MS-DRG 91, 92, 93, with relative weights from 0.7783 to 1.7050 depending on complications. Higher weights mean higher Medicare reimbursement.
Can G71.3 be reported with H49.81?
Not as a rule. The Excludes1 note on G71.3 lists "Kearns-Sayre syndrome (H49.81)". 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 is the ICD-9 equivalent of G71.3?
Under the General Equivalence Mappings, mitochondrial myopathy, not elsewhere classified converts to ICD-9-CM 359.89 (myopathies NEC). The mapping is approximate, so confirm the match fits the documentation.
Does G71.3 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage: G71.3 maps to no category in the CMS-HCC V28 model. It still risk-adjusts in the ESRD (V21) category 75 (Polyneuropathy).
