ICD-10-CM 2027
ICD-10 code for Barth syndrome
From the official ICD-10-CM alphabetic index entry “Barth syndrome”. Page updated September 29, 2026.
About coding Barth syndrome
The ICD-10-CM code for Barth syndrome is E78.71 (Barth syndrome).
Within E78.7 (disorders of bile acid and cholesterol metabolism), E78.71 is specifically for Barth syndrome. Related codes cover disorder of bile acid and cholesterol metabolism, unspecified (E78.70), Smith-Lemli-Opitz syndrome (E78.72) and other disorders of bile acid and cholesterol metabolism (E78.79).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 564 (Other Musculoskeletal System and Connective Tissue Diagnoses with MCC, relative weight 1.4908), DRG 565 (Other Musculoskeletal System and Connective Tissue Diagnoses with CC, relative weight 0.9783) and DRG 566 (Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC, relative weight 0.7378), in MDC 08 (Diseases and Disorders of the Musculoskeletal System and Connective Tissue), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Barth syndrome ICD-10 codes
What is the ICD-10 code for Barth syndrome?
- E78.71 — Barth syndrome.
Is E78.71 billable?
- Yes. E78.71 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.71 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.71 as a principal diagnosis.
Is E78.71 a CC or MCC?
- E78.71 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.