ICD-10 Code E71.310 – Long chain/very long chain acyl CoA dehydrogenase deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.310 – Long chain/very long chain acyl CoA dehydrogenase deficiency
What it is
E71.310 identifies a hereditary disorder of fatty acid oxidation caused by deficiency of long-chain or very-long-chain acyl-CoA dehydrogenase. It affects the body’s ability to use certain fats for energy, especially during fasting or illness.
Clinical signs
Clinical features vary; refer to documentation. Commonly documented findings include recurrent hypoglycemia, muscle weakness, cardiomyopathy, liver dysfunction, or episodes triggered by fasting, infection, or prolonged exercise.
When to use this code
Use this code when the record clearly documents long chain/very long chain acyl CoA dehydrogenase deficiency, including confirmed genetic or metabolic diagnoses. It may also apply when the provider specifies VLCAD/LCAD deficiency or a related inherited fatty acid oxidation disorder. Check documentation if the wording is unclear.
Do not use for
Do not use this code for nonspecific hypoglycemia, muscle pain, or liver disease without a documented fatty acid oxidation defect. Do not assign it if the chart names a different metabolic disorder.
Coding tip
Confirm the exact enzyme deficiency in the assessment or problem list before coding, and query the provider when the diagnosis is abbreviated or ambiguous.