2026 ICD-10-CM Diagnosis Code E78.71Barth syndrome

ICD-10-CM Codes›E00–E89›E70-E88›E78

ICD-10-CM E78.71
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E78.71 is a billable ICD-10-CM diagnosis code for barth syndrome. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 564 through 566. Coders also document this condition as 3-Methylglutaconic aciduria type 2. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E78.71
Billable Status
Yes — Valid for Submission
Code Describes
Barth syndrome
Short Description
Barth syndrome
Same as the full description in the CMS dataset.
Parent Code
E78.7
Disorders of bile acid and cholesterol metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE78Disorders of lipoprotein metabolism and other lipidemias
This CodeE78.71Barth syndrome

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 3-Methylglutaconic aciduria type 2

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Barth syndrome
    • Disorder(of)
      • bile acid and cholesterol metabolism
        • Barth syndrome
    • Disorder(of)
      • cholesterol and bile acid metabolism
        • Barth syndrome
    • Syndrome
      • Barth

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Barth Syndrome

    rare congenital x-linked disorder of lipid metabolism. barth syndrome is transmitted in an x-linked recessive pattern. the syndrome is characterized by muscular weakness, growth retardation, dilated cardiomyopathy, variable neutropenia, 3-methylglutaconic aciduria (type ii) and decreases in mitochondrial cardiolipin level. other biochemical and morphological mitochondrial abnormalities also exist.

Patient EducationClinical

Genetic Brain Disorders

A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.

Read the full article at MedlinePlus

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Convert E78.71 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
759.89 Specfied cong anomal NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E78.71Overview

Is E78.71 (Disorders of bile acid and cholesterol metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report barth syndrome on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does E78.71 group to?

When barth syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 565, 566, with relative weights from 0.7493 to 1.5436 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of E78.71?

Under the General Equivalence Mappings, barth syndrome converts to ICD-9-CM 759.89 (specfied cong anomal NEC). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[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.