ICD-10 Coding for Familial Hypercholesterolemia(E78.01, Z83.42)
Complete ICD-10-CM coding and documentation guide for Familial Hypercholesterolemia. Includes clinical validation requirements, documentation requirements, and coding pitfalls.
Also known as:
Related ICD-10 Code Ranges
Complete code families applicable to Familial Hypercholesterolemia
E78.0-E78.9Primary Range
Disorders of lipoprotein metabolism and other lipidemias
This range includes all hyperlipidemia disorders, with E78.01 specifically for familial hypercholesterolemia.
Family history of other specific disorders
Includes family history of hyperlipidemia, specifically Z83.42 for familial hypercholesterolemia.
Code Comparison: When to Use Each Code
Compare key differences between these codes to ensure accurate selection
| Code | Description | When to Use | Key Documentation |
|---|---|---|---|
| E78.01 | Familial hypercholesterolemia | Use when a patient has a confirmed diagnosis of familial hypercholesterolemia through genetic testing or clinical criteria. |
|
| Z83.42 | Family history of familial hypercholesterolemia | Use when there is a documented family history of FH impacting patient management. |
|
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 hypercholesterolemia
Essential facts and insights about Familial Hypercholesterolemia
Primary ICD-10-CM Codes for familial hypercholesterolemia
Decision Criteria
clinical Criteria
- LDL-C ≥190 mg/dL and genetic variant confirmed
documentation Criteria
- Documented family history of FH with genetic confirmation
Applicable To
- Genetic hypercholesterolemia
- Heterozygous familial hypercholesterolemia
Excludes
- Secondary hyperlipidemia (E78.2)
Clinical Validation Requirements
- LDL-C ≥190 mg/dL
- Genetic testing confirming LDLR, APOB, or PCSK9 variant
- Presence of tendon xanthomas
Code-Specific Risks
- Incorrectly coding as unspecified hyperlipidemia (E78.00)
- Missing documentation of genetic confirmation
Coding Notes
- Ensure genetic testing results and LDL-C levels are documented to support the use of E78.01.
Z83.42 - Family history of familial hypercholesterolemia
Use when there is a documented family history of FH impacting patient management.
Applicable To
- Family history of genetic hypercholesterolemia
Excludes
- Personal history of hyperlipidemia (E78.0-E78.9)
Clinical Validation Requirements
- Documented family history of FH in first or second-degree relatives
Coding Notes
Ensure family history is clearly documented, including the specific relative and their diagnosis.
Ancillary Codes
Additional codes that should be used in conjunction with the main diagnosis codes when applicable.
Family history of familial hypercholesterolemia
Z83.42Documentation & Coding Risks
Avoid these common documentation and coding issues when documenting Familial Hypercholesterolemia to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code E78.01.
Impact
Clinical: May affect risk assessment and management strategies, Regulatory: Non-compliance with coding standards, Financial: Potential for denied claims due to incomplete documentation
Mitigation Strategy
Ensure thorough family history is taken and documented, Use templates to capture all relevant information
Impact
Reimbursement: May lead to lower reimbursement rates, Compliance: Non-compliance with coding guidelines, Data Quality: Inaccurate patient records and statistics
Mitigation Strategy
Confirm genetic testing results and document family history to support E78.01.
Impact
Failure to document genetic testing results can lead to coding errors.
Mitigation Strategy
Implement checklists to ensure all genetic testing results are documented.
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 Hypercholesterolemia, with expert answers to help guide accurate code selection and documentation.
Documentation Templates for Familial Hypercholesterolemia
Use these documentation templates to ensure complete and accurate documentation for Familial Hypercholesterolemia. These templates include all required elements for proper coding and billing.
Initial Diagnosis of Familial Hypercholesterolemia
Specialty: CardiologyRequired Elements
- LDL-C levels
- Genetic testing results
- Family history details
- Physical examination findings
Example Documentation
Examples: Poor vs. Good Documentation
Poor Documentation Example
Good Documentation Example
Explanation
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