E78.71Barth syndrome

E78.71 is the ICD-10-CM diagnosis code for Barth syndrome. It belongs to category E78 (disorders of lipoprotein metabolism and other lipidemias), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

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.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 759.89 (other specified congenital anomalies).