2027 ICD-10-CM Diagnosis Code E71.311Medium chain acyl CoA dehydrogenase deficiency
ICD-10-CM Codes›E00–E89›E70-E88›E71
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
E71.311 is a billable ICD-10-CM diagnosis code for medium chain acyl CoA dehydrogenase deficiency. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 39 closely related codes. Coders also document this condition as Acyl-CoA dehydrogenase deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
E71.311 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 23 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 43 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E71.311 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Acyl-CoA dehydrogenase deficiency
- Medium-chain acyl-coenzyme A dehydrogenase deficiency
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E71.311: its own notes plus those printed at E71.3, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- MCAD deficiency
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- carnitine deficiency due to inborn error of metabolism (E71.42)
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E71.311, its category, or a range that includes it.
Excludes1 4
These codes carry an Excludes1 note naming E71.311: they are not reported together with it, unless the two conditions are unrelated.
- E72 Other disorders of amino-acid metabolismfatty-acid metabolism (E71.3) names E71.3, which includes this code
- R79 Other abnormal findings of blood chemistry
- R79.83 Abnormal findings of blood amino-acid level
- Block R80-R82 Abnormal findings on examination of urine, without diagnosis
Index to Diseases and InjuriesGuidance
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Medium-Chain Acyl-CoA Dehydrogenase Deficiency
a genetic disorder characterized by deficiency of the enzyme medium-chain acyl-coenzyme a dehydrogenase that metabolizes medium-chain fatty acids. signs and symptoms appear in infancy or childhood and may be triggered during fasting or illness. they include vomiting, hypoglycemia and lethargy.
Patient EducationClinical
Lipid Metabolism Disorders
Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E71.311 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E71.311Overview
What is the ICD-10 code for medium chain acyl CoA dehydrogenase deficiency?
The ICD-10-CM code for medium chain acyl CoA dehydrogenase deficiency is E71.311 (sometimes written as E71311). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E71.311 (Disorders of fatty-acid oxidation) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report medium chain acyl CoA dehydrogenase deficiency on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E71.311 group to?
When medium chain acyl CoA dehydrogenase deficiency is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Is E71.311 a CC or MCC?
CMS lists E71.311 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 39 closely related codes in its exclusion list.
Can E71.311 be reported with E71.5?
Not as a rule. The Excludes1 note at E71.3, which applies to E71.311, lists "peroxisomal disorders (E71.5)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
Does E71.311 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E71.311 mapped to HCC 23 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders).
