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.

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

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. E71.314 was a payment HCC under V24 (Other Significant Endocrine and Metabolic Disorders) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

What should coders know when reporting 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 Because E71.314 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

E71.314: Muscle carnitine palmitoyltransferase

ICD-10-CM Code View

HCC Buddy Code Card

Digital ICD-10 code-book layout with official code detail, always-visible risk models, Code Trumping, and Buddy coding guidance.

Code lookupE71.314Effective 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

CMS-HCC V28

Not risk-adjusted for this model/period.

RAF 0

CMS-HCC V24

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.230

ACA/HHS

Not risk-adjusted for this model/period.

RAF 0

ESRD/PACE

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.0

RXHCC

Mapped

HCC 43

Other Significant Endocrine a...

RAF 0.0

Code Trumping

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Code Book Path

Official

E71.3Disorders of fatty-acid metabolism

E71.31Disorders of fatty-acid oxidation

E71.314Muscle carnitine palmitoyltransferase deficiency

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E71.314 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E71.314 in this effective period.

Related Child 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

ICD-10-CM does not list Includes notes for E71.314 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E71.314 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E71.314 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E71.314 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E71.314 in this effective period.

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.

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is E71.314 an HCC code?

Yes. E71.314 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

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

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

MEAT Criteria for E71.314

For E71.314to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

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

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E71.314 during that encounter, not just copy-forwarded from a problem list.

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).

Under the older CMS-HCC V24 model, E71.314 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Document whether this affects skeletal muscle, cardiac muscle, or both, as clinical presentation varies. Because E71.314 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, 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.