E78.71 Barth syndrome

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applies from E78 Disorders of lipoprotein metabolism and other lipidemias

Excludes1 (never code together)

• sphingolipidosis (E75.0-E75.3)

Applies from E78.7 Disorders of bile acid and cholesterol metabolism

Excludes1 (never code together)

• Niemann-Pick disease type C (E75.242)

MS-DRG index

Defines the logic of DRG(s) 564-566 in MDC 08.

Approximate ICD-9-CM (GEMs)

• 759.89 Other specified congenital anomalies (approximate)

Code annotations containing back-references to E78.71

Back-references to E78

• Type 2 Excludes: E88.0 Disorders of plasma-protein metabolism, not elsewhere classified ("disorder of lipoprotein metabolism (E78.-)")
• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")

Diagnosis Index entries for E78.71

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

Nearby codes

• E78.49 Other hyperlipidemia
• E78.5 Hyperlipidemia, unspecified
• E78.6 Lipoprotein deficiency
• E78.7 Disorders of bile acid and cholesterol metabolism
• E78.70 Disorder of bile acid and cholesterol metabolism, unspecified
• E78.71 Barth syndrome (this code)
• E78.72 Smith-Lemli-Opitz syndrome
• E78.79 Other disorders of bile acid and cholesterol metabolism
• E78.8 Other disorders of lipoprotein metabolism
• E78.81 Lipoid dermatoarthritis
• E78.89 Other lipoprotein metabolism disorders