E71.310

HCC Category Mapping

What This Code Means

This is a rare inherited metabolic disorder where the body cannot properly break down certain fatty acids, leading to a buildup of toxic substances in the blood and tissues. This condition can cause muscle weakness, liver problems, and heart issues, especially during illness or fasting.

Coding Tips

• •This is a specific subtype of fatty acid oxidation disorder - ensure you are not using the parent code E71.31 unless the specific type is truly unspecified
• •Document any associated complications (cardiomyopathy, hepatomegaly, rhabdomyolysis) with additional codes, as this metabolic disorder often presents with multi-system involvement

Clinical Significance

Long chain and very long chain acyl-CoA dehydrogenase deficiency (LCAD/VLCAD) impairs the body's ability to oxidize long-chain fatty acids for energy, particularly during fasting or physiological stress. This condition carries significant risk of hypoketotic hypoglycemia, rhabdomyolysis, cardiomyopathy, and sudden death, especially in infancy. Newborn screening has improved early detection, but lifelong dietary management with medium-chain triglyceride supplementation is required.

Documentation Requirements

• ✓Document the specific chain-length deficiency (LCAD vs.
• ✓VLCAD), newborn screening results or confirmatory enzyme/genetic testing, acylcarnitine profiles, cardiac function assessments, and dietary management plan.
• ✓Record any episodes of rhabdomyolysis, hypoglycemia, or cardiac complications.

Commonly Confused Codes