E71.41 ICD-10-CM Code: Primary carnitine deficiency
E71.41 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · free HCC coding tools
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.41
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidancePrimary carnitine deficiency
A rare genetic condition where the body cannot produce enough carnitine, a substance essential for converting fats into energy, due to inherited genetic mutations.
Buddy Insight
Primary carnitine deficiency (systemic carnitine deficiency) is caused by mutations in the SLC22A5 gene encoding the carnitine transporter OCTN2, resulting in excessive urinary carnitine loss and critically low plasma carnitine levels.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
MappedHCC 23
RAF 0.194
ACA/HHS
MappedHCC 29
Varies by metal level
ESRD/PACE
MappedHCC 23
RAF 0.036
RXHCC
MappedHCC 43
RAF 0.063
Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for E71.41 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E71.41 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E71.41 in this effective period.
Excludes 1
Official- Muscle carnitine palmitoyltransferase deficiency (E71.314)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E71.41 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E71.41 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E71.41 in this effective period.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
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 E71.41 an HCC code?
Yes. E71.41 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.
- Code
- E71.41
- Description
- Primary carnitine deficiency
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF
- —
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
Each model's RAF is its CMS base weight for that model's standard population, so 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 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.
Work E71.41 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E71.41
For E71.41 to 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 E71.41 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E71.41 is the ICD-10-CM diagnosis code for primary carnitine deficiency. A rare genetic condition where the body cannot produce enough carnitine, a substance essential for converting fats into energy, due to inherited genetic mutations. E71.41 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, E71.41 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.194. 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 is systemic carnitine deficiency or muscle-specific carnitine deficiency. Because E71.41 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 E71.41 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Document whether this is systemic carnitine deficiency or muscle-specific carnitine deficiency
- •Note any cardiac involvement (cardiomyopathy) or muscle weakness that may require additional codes
Clinical Significance
Primary carnitine deficiency (systemic carnitine deficiency) is caused by mutations in the SLC22A5 gene encoding the carnitine transporter OCTN2, resulting in excessive urinary carnitine loss and critically low plasma carnitine levels. Without treatment, patients face risk of hypoketotic hypoglycemia, cardiomyopathy, and sudden death. Lifelong oral carnitine supplementation is highly effective and dramatically improves outcomes.
Documentation Requirements
- ✓Document plasma carnitine levels (typically very low free carnitine <5 micromol/L), genetic testing confirming SLC22A5 mutations, echocardiogram findings, clinical presentation, and carnitine supplementation dosage and response.
- ✓Record whether identified through newborn screening or clinical presentation.