What is ICD-10 code E71.314?

E71.314 is the ICD-10-CM diagnosis code for Muscle carnitine palmitoyltransferase deficiency. A genetic condition affecting muscle function where the enzyme carnitine palmitoyltransferase is deficient, preventing the body from properly using fats for energy in muscle tissue. E71.314 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E71.314 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. E71.314 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. E71.314 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

Which HCC does E71.314 map to under V28?

E71.314 has no payable CMS-HCC V28 mapping in the final FY2026 CMS file.

What RAF reference is published for E71.314?

E71.314 has no V28 reference coefficient because the final FY2026 source verifies no payable V28 mapping.

What is the V28 vs V24 difference for E71.314?

E71.314 has verified no payable V28 mapping in the final FY2026 source. Select a historical effective period before making any V24 comparison.

What documentation supports E71.314?

Document whether this affects skeletal muscle, cardiac muscle, or both, as clinical presentation varies Note any episodes of rhabdomyolysis, myoglobinuria, or acute muscle weakness that may require additional codes E71.314 is not a CMS-HCC V28 payment code in the verified current source, so MEAT is not the payment hook. Documentation still has to support the diagnosis if you report it.

Can E71.314 be a primary diagnosis?

Yes. E71.314 carries no code-first instruction in the FY2026 tabular, so it can be the first-listed diagnosis when it is the condition chiefly responsible for the encounter and the note supports it, and it can also be reported as a secondary diagnosis. For risk adjustment the order does not matter; the V28 category is the same either way.

Which codes cannot be reported with E71.314?

The Excludes1 note that applies to E71.314 lists transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); hemolytic anemias attributable to enzyme disorders (D55.-); and 5 more (see the notes on this page). An Excludes1 condition is not reported together with E71.314; report one or the other, unless the two conditions are unrelated, which the ICD-10-CM Official Guidelines (Section I.A.12.a) allow. The Excludes2 note lists Ehlers-Danlos syndromes (Q79.6-); carnitine deficiency due to inborn error of metabolism (E71.42); those are separate conditions that may be reported together with E71.314 when both are documented.

2026 ICD-10-CM E71.314: Muscle carnitine

E71.314 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · free HCC coding tools

ICD-10-CM Code View

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Code lookupE71.314

Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E71.314

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Muscle carnitine palmitoyltransferase deficiency

A genetic condition affecting muscle function where the enzyme carnitine palmitoyltransferase is deficient, preventing the body from properly using fats for energy in muscle tissue.

Buddy Insight

Overview Risk Models Code Book Documentation Audit Notes Edition comparison

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Historical

Not used for standard CY2026 Medicare Advantage payment. Select a historical period to review V24.

Not used for CY2026 payment

ACA/HHS

Context needed

HCC 028

Conditional HHS-HCC mapping: HCC 028 when age < 21. Select the member context before showing a coefficient reference. Metal level and model segment are required for coefficients; no single RAF is shown.

Coefficient needs member context

ESRD/PACE

Context needed

HCC 23

ESRD candidates: HCC 23 when age < 21 or age >= 21. ESRD mapping is separate from PACE. Dialysis, graft, age, and other member context determine the applicable coefficient.

Coefficient needs member context

RXHCC

Mapped

HCC 43

Pituitary, Adrenal Gland, and...

Code-level coefficient reference

Code Book Path

Official

E71.3Disorders of fatty-acid metabolism E71.31Disorders of fatty-acid oxidation E71.314Muscle carnitine palmitoyltransferase deficiency

Inclusion Terms

Official

No inclusion terms are included in this display for E71.314. Check the code and parent instructions in the Code Book.

Excludes 2

Official

Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88, E71.3
carnitine deficiency due to inborn error of metabolism (E71.42)Inherited from E70-E88, E71.3

Related Codes

Official

E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency E71.311Medium chain acyl CoA dehydrogenase deficiency E71.312Short chain acyl CoA dehydrogenase deficiency E71.313Glutaric aciduria type II E71.318Other disorders of fatty-acid oxidation

Includes

Official

No Includes notes are included in this display for E71.314. Check the code and parent instructions in the Code Book.

Excludes 1

Official

transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88, E71.3
androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88, E71.3
congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88, E71.3
hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88, E71.3
Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88, E71.3
5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88, E71.3
peroxisomal disorders (E71.5)Inherited from E00-E89, E70-E88, E71.3
Refsum's disease (G60.1)Inherited from E00-E89, E70-E88, E71.3
Schilder's disease (G37.0)Inherited from E00-E89, E70-E88, E71.3

Code First

Official

No Code First sequencing instructions are included in this display for E71.314. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for E71.314. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for E71.314. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance

Document which CPT isoform is deficient (CPT I vs CPT II), whether the presentation is myopathic, infantile, or neonatal, creatine kinase levels during episodes, genetic testing results, history of rhabdomyolysis episodes, and dietary management plan.

Record any cardiac or hepatic involvement.

MEAT Support

HCC Buddy guidance

Record any cardiac or hepatic involvement.

Audit Caution

HCC Buddy guidance

Ensure documentation specifies CPT deficiency affecting muscle specifically.

Do not code rhabdomyolysis episodes under myopathy codes without identifying the underlying CPT deficiency as the root cause.

Common Mistakes

HCC Buddy guidance

E71.310 (Long chain/very long chain acyl CoA dehydrogenase deficiency) which also presents with rhabdomyolysis but involves a different enzyme

E71.318 (Other disorders of fatty-acid oxidation) for non-specific oxidation defects

G72.89 (Other specified myopathies) when the metabolic basis is not recognized.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is E71.314 an HCC code?

E71.314 is not in the CMS-HCC V28 or V24 community payment model. E71.314 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E71.314 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping.

Code: E71.314
Description: Muscle carnitine palmitoyltransferase deficiency
HCC (V28): No CMS-HCC V28 mapping
RAF reference coefficient: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

RxHCCHCC 43, Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders

Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work E71.314 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for E71.314

For E71.314, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
EEvaluate: test results, medication response, or physical findings reviewed by the provider
AAssess: explicit mention in the assessment or plan with acknowledgment of status
TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Coder workflow notes

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What This Code Means

E71.314 is the ICD-10-CM diagnosis code for muscle carnitine palmitoyltransferase deficiency. A genetic condition affecting muscle function where the enzyme carnitine palmitoyltransferase is deficient, preventing the body from properly using fats for energy in muscle tissue. E71.314 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

E71.314 has no mapping under the CMS-HCC V28 or V24 community payment models. E71.314 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E71.314 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.

Document whether this affects skeletal muscle, cardiac muscle, or both, as clinical presentation varies.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E71.314 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Document whether this affects skeletal muscle, cardiac muscle, or both, as clinical presentation varies
•Note any episodes of rhabdomyolysis, myoglobinuria, or acute muscle weakness that may require additional codes

Clinical Significance

Muscle carnitine palmitoyltransferase (CPT II) deficiency is the most common inherited disorder of lipid metabolism affecting skeletal muscle, presenting with recurrent episodes of rhabdomyolysis, myoglobinuria, and muscle pain triggered by prolonged exercise, fasting, or illness. The myopathic form typically presents in adolescence or adulthood, while severe infantile forms can cause cardiomyopathy and liver failure. Long-term management focuses on dietary modification and avoidance of metabolic triggers.

Documentation Requirements

✓Document which CPT isoform is deficient (CPT I vs CPT II), whether the presentation is myopathic, infantile, or neonatal, creatine kinase levels during episodes, genetic testing results, history of rhabdomyolysis episodes, and dietary management plan.
✓Record any cardiac or hepatic involvement.

Commonly Confused Codes

•E71.310 (Long chain/very long chain acyl CoA dehydrogenase deficiency) which also presents with rhabdomyolysis but involves a different enzyme
•E71.318 (Other disorders of fatty-acid oxidation) for non-specific oxidation defects
•G72.89 (Other specified myopathies) when the metabolic basis is not recognized.