2027 ICD-10-CM Diagnosis Code E78.71Barth syndrome
ICD-10-CM Codes›E00–E89›E70-E88›E78
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
E78.71 is a billable ICD-10-CM diagnosis code for barth syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 564 through 566. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 42 closely related codes. 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
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- 3-Methylglutaconic aciduria type 2
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E78.71: its own notes plus those printed at E78.7, E78, block E70-E88, and Chapter 4. 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.
- Niemann-Pick disease type C (E75.242)
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.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- 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.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E78.71, its category, or a range that includes it.
Excludes2 2
These codes carry an Excludes2 note naming E78.71: its condition is not part of those codes, and both may be reported when the patient has both.
- E88.0 Disorders of plasma-protein metabolism, not elsewhere classifieddisorder of lipoprotein metabolism (E78.-) names E78.-, which includes this code
- Block R70-R79 Abnormal findings on examination of blood, without diagnosisabnormalities of lipids (E78.-) names E78.-, which includes this code
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Barth syndrome E78.71
Disorder (of) See Also: Disease;
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E78.71 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E78.71Overview
What is the ICD-10 code for barth syndrome?
The ICD-10-CM code for barth syndrome is E78.71 (sometimes written as E7871). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
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, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.
Is E78.71 a CC or MCC?
CMS lists E78.71 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 42 closely related codes in its exclusion list.
Can E78.71 be reported with E75.242?
Not as a rule. The Excludes1 note at E78.7, which applies to E78.71, lists "Niemann-Pick disease type C (E75.242)". 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).
