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E78.71

Billable

Barth syndrome

This code does not map to an HCC category in any model (V28, V24, ESRD, RxHCC).

What This Code Means

Barth syndrome is a rare inherited genetic disorder affecting how the body processes lipids, causing muscle weakness, heart problems, and immune deficiencies.

Coding Tips

  • •This is an X-linked genetic disorder; document family history and genetic testing confirmation in the record
  • •Barth syndrome primarily affects males; verify patient gender and clinical presentation

Code Hierarchy

└E78Disorders of lipoprotein metabolism and other lipidemias└E78.7Disorders of bile acid and cholesterol metabolism└E78.71Barth syndrome
└E78.71Barth syndrome

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