ICD-10 Code for Elevated Cholesterol
The ICD-10 code for hypercholesterolemia (elevated cholesterol) is E78.0 for pure hypercholesterolemia, or E78.00 for unspecified hypercholesterolemia. 1
Specific ICD-10 Coding Categories
Pure Hypercholesterolemia (E78.0)
- E78.00: Pure hypercholesterolemia, unspecified - used for general elevated cholesterol without further specification 2, 3
- E78.01: Familial hypercholesterolemia (heterozygous FH) - used when LDL-C ≥190 mg/dL (5 mmol/L) for adults or ≥160 mg/dL (4 mmol/L) for children with a first-degree relative similarly affected or with premature coronary artery disease 1
Familial Hypercholesterolemia Subcategories
- Heterozygous FH (E78.01): Requires LDL-C ≥190 mg/dL for adults and ≥160 mg/dL for children, plus either a first-degree relative with similar findings, premature CAD, or positive genetic testing for LDL-C-raising gene defects (LDL receptor, apoB, or PCSK9) 1
- Homozygous FH: Diagnosed when LDL-C ≥400 mg/dL (10 mmol/L) with one or both parents having clinically diagnosed FH, or with positive genetic testing showing two identical or nonidentical abnormal LDL-raising gene defects 1
Mixed Hyperlipidemia (E78.2)
- Used when patients have elevated LDL-C above their goal and either triglycerides >150 mg/dL or HDL-C <40 mg/dL (males) or <50 mg/dL (females) 4
- Approximately 40% of patients initially coded as having simple hypercholesterolemia actually meet criteria for mixed hyperlipidemia 4
Clinical Context for Coding
When to Use E78.00 vs E78.01
- Use E78.00 for general elevated cholesterol without family history or genetic confirmation 2, 3
- Use E78.01 when clinical criteria for familial hypercholesterolemia are met, even without genetic testing, based on LDL-C thresholds and family history 1
- Genetic testing is not required for coding E78.01 if clinical criteria are satisfied 1
Important Coding Considerations
- The ICD-10 code E78.01 for familial hypercholesterolemia was not available until 2016, so older records may use alternative coding systems like SNOMED 3
- When using claims data or electronic health records, the sensitivity of E78.01 coding is 84.4% and specificity is 99.4% compared to clinical diagnostic criteria, but positive predictive value is only 6.4%, indicating significant undercoding 3
- Many patients with atherosclerosis (ICD-9 codes 440.xx, 414.x, 437.x) have concurrent hypercholesterolemia that should be separately coded 5
Common Pitfalls
- Avoid undercoding mixed hyperlipidemia: Many patients coded as E78.0 actually have E78.2 (mixed hyperlipidemia) when triglycerides and HDL-C are considered 4
- Don't overlook familial hypercholesterolemia: Patients with LDL-C ≥190 mg/dL should be evaluated for E78.01 coding, as this affects treatment intensity and family screening recommendations 1
- Document baseline lipid values: Approximately 62% of patients with atherosclerosis lack documented baseline LDL-C values, which impairs appropriate coding and treatment monitoring 5