E71.40

Billable

Disorder of carnitine metabolism, unspecified

HCC Category Mapping

V24HCC 23 — Other Significant Endocrine and Metabolic Disorders

0.230

ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders

0.000

RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders

0.000

What This Code Means

A metabolic disorder affecting carnitine, a substance the body needs to convert fats into energy, where the specific cause or type has not been determined.

Coding Tips

•This unspecified code should be used only when the type of carnitine deficiency cannot be determined from documentation
•Attempt to obtain additional information to assign a more specific code (E71.41, E71.42, or E71.43)

Clinical Significance

Unspecified carnitine metabolism disorder indicates a defect in carnitine homeostasis where the body cannot properly synthesize, transport, or utilize carnitine for fatty acid transport into mitochondria. Carnitine deficiency can cause muscle weakness, cardiomyopathy, and hepatic dysfunction. Determining whether the deficiency is primary, secondary, or iatrogenic is important for targeted treatment.

Documentation Requirements

✓Document plasma carnitine levels (free and total), acylcarnitine profile, clinical symptoms, and reason why the specific type of carnitine metabolism disorder has not been determined.
✓Record any cardiac or muscle involvement and current carnitine supplementation status.

Commonly Confused Codes

E71.41 (Primary carnitine deficiency) when genetic carnitine transporter defect is confirmed E71.42 (Carnitine deficiency due to inborn errors of metabolism) for secondary genetic causes E71.43 (Iatrogenic carnitine deficiency) when medication or treatment is the cause.

Code Hierarchy

└E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism └E71.4Disorders of carnitine metabolism └E71.40Disorder of carnitine metabolism, unspecified

└E71.40Disorder of carnitine metabolism, unspecified