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ICD-10 Coding for Familial Hyperlipidemia(E78.01, Z83.42)

Complete ICD-10-CM coding and documentation guide for Familial Hyperlipidemia. Includes clinical validation requirements, documentation requirements, and coding pitfalls.

Also known as:

Familial HypercholesterolemiaGenetic Hyperlipidemia

Related ICD-10 Code Ranges

Complete code families applicable to Familial Hyperlipidemia

E78.0-E78.5Primary Range

Disorders of lipoprotein metabolism and other lipidemias

This range includes codes for various lipid metabolism disorders, with E78.01 specifically for familial hypercholesterolemia.

Code Comparison: When to Use Each Code

Compare key differences between these codes to ensure accurate selection

CodeDescriptionWhen to UseKey Documentation
E78.01Familial hypercholesterolemiaUse when familial hypercholesterolemia is confirmed by genetic testing or clinical criteria.
  • LDL-C ≥190 mg/dL in adults
  • Genetic testing confirming LDLR/APOB/PCSK9 mutation
  • Presence of tendon xanthomas
Z83.42Family history of familial hypercholesterolemiaUse when documenting family history of FH without a confirmed diagnosis in the patient.
  • Documented family history of FH in first-degree relatives

Clinical Decision Support

Always review the patient's clinical documentation thoroughly. When in doubt, choose the more specific code and ensure documentation supports it.

Key Information: ICD-10 code for familial hyperlipidemia

Essential facts and insights about Familial Hyperlipidemia

The ICD-10 code for familial hyperlipidemia is E78.01, indicating confirmed familial hypercholesterolemia.

Primary ICD-10-CM Codes for familial hyperlipidemia

Familial hypercholesterolemia
Billable Code

Decision Criteria

clinical Criteria

  • LDL-C levels and genetic test results

documentation Criteria

  • Specific mention of familial hypercholesterolemia

Applicable To

  • Genetic hyperlipidemia
  • LDL receptor defect

Excludes

  • Secondary hyperlipidemia (E78.2)

Clinical Validation Requirements

  • LDL-C ≥190 mg/dL in adults
  • Genetic testing confirming LDLR/APOB/PCSK9 mutation
  • Presence of tendon xanthomas

Code-Specific Risks

  • Misclassification if genetic testing is not documented
  • Incorrect sequencing with comorbid conditions

Coding Notes

  • Ensure documentation specifies familial hypercholesterolemia and includes genetic or clinical validation.

Ancillary Codes

Additional codes that should be used in conjunction with the main diagnosis codes when applicable.

Family history of familial hypercholesterolemia

Z83.42
Use when there is a family history of FH but the patient is not diagnosed.

Differential Codes

Alternative codes to consider when ruling out similar conditions to the primary diagnosis.

Unspecified hyperlipidemia

E78.5
Use E78.5 when hyperlipidemia is documented without familial or genetic context.

Documentation & Coding Risks

Avoid these common documentation and coding issues when documenting Familial Hyperlipidemia to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code E78.01.

Impact

Clinical: Inaccurate risk assessment for cardiovascular events, Regulatory: Potential audit issues, Financial: Loss of reimbursement opportunities

Mitigation Strategy

Always document family history when relevant, Use templates to ensure completeness

Impact

Reimbursement: Potential underpayment due to incorrect coding, Compliance: Non-compliance with coding guidelines, Data Quality: Inaccurate clinical data representation

Mitigation Strategy

Ensure documentation supports E78.01 with genetic or clinical criteria.

Impact

Using E78.5 instead of E78.01 for familial cases

Mitigation Strategy

Educate coders on the importance of genetic and clinical validation.

Documentation errors, coding pitfalls, and audit risks are interconnected aspects of medical coding and billing. Addressing all three areas helps ensure accurate coding, optimal reimbursement, and regulatory compliance.

Frequently Asked Questions

Common questions about ICD-10 coding for Familial Hyperlipidemia, with expert answers to help guide accurate code selection and documentation.

Documentation Templates for Familial Hyperlipidemia

Use these documentation templates to ensure complete and accurate documentation for Familial Hyperlipidemia. These templates include all required elements for proper coding and billing.

Confirmed Familial Hypercholesterolemia

Specialty: Cardiology

Required Elements

  • Family history of premature CAD
  • LDL-C levels
  • Genetic test results
  • Physical exam findings

Example Documentation

Patient presents with LDL-C 250 mg/dL, family history of MI in father at age 45. Physical exam reveals xanthomas on Achilles tendons. Diagnosed with familial hypercholesterolemia (E78.01).

Examples: Poor vs. Good Documentation

Poor Documentation Example
Patient has high cholesterol with family history.
Good Documentation Example
Patient diagnosed with familial hypercholesterolemia (E78.01) based on LDL-C 220 mg/dL and family history of MI.
Explanation
The good example specifies the diagnosis and provides clinical validation.

Need help with ICD-10 coding for Familial Hyperlipidemia? Ask your questions below.

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