ICD 10 Codes From A30-A49
January 16, 2026
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When it comes to keeping accurate medical records, knowing the right ICD-10 codes for family history of high cholesterol and related conditions is crucial. This guide breaks down the important diagnosis codes you’ll need when documenting familial hypercholesterolemia and a family history of hyperlipidemia in patient charts.
Familial hypercholesterolemia (FH) is a genetic disorder that causes dangerously high levels of low-density lipoprotein (LDL) cholesterol from birth. This inherited condition significantly increases the risk of early heart disease and cardiovascular complications. When patients have a family history of high cholesterol or hyperlipidemia, proper documentation using the correct ICD-10 codes ensures appropriate treatment planning and insurance reimbursement.
The Centers for Disease Control and Prevention provides valuable resources on cholesterol management and heart disease prevention that healthcare providers can reference when treating patients with these conditions.
The ICD-10 code for familial hypercholesterolemia is E78.01. This specific code falls under the category of endocrine, nutritional, and metabolic diseases. It represents pure hypercholesterolemia that is inherited and clearly identifies the condition as a genetic disorder rather than lifestyle-related high cholesterol.
Key characteristics of E78.01:
When documenting heterozygous familial hypercholesterolemia, the ICD-10 code remains E78.01. This code covers both heterozygous (one affected gene) and homozygous (two affected genes) forms of the condition, though the severity typically differs between these variants.
While there isn’t a single dedicated code specifically labeled “family history of hypercholesterolemia ICD-10,” healthcare providers use Z83.42 for documenting family history of elevated lipoprotein(a) and related lipid disorders. This code belongs to the Z-codes category representing factors influencing health status and contact with health services.
The ICD-10 code for family history of hyperlipidemia is Z83.49. This code serves as a broader category for documenting:
This is a billable, non-specific code that medical coders use when documenting a patient’s personal history and family health background. The code helps identify patients at increased risk who may benefit from earlier screening or preventive interventions.
When a patient has been diagnosed with familial hypercholesterolemia through genetic testing or clinical criteria, use E78.01 as the primary diagnosis code. This applies whether the patient has:
When documenting that a patient has relatives with high cholesterol or hyperlipidemia but doesn’t have the condition themselves, use the appropriate Z-code:
These family history codes help identify at-risk individuals who may need:
For accurate coding and optimal patient care, healthcare providers should document:
Specific family relationships: Note whether the family history involves first-degree relatives (parents, siblings, children) or second-degree relatives (grandparents, aunts, uncles)
Age of onset: Document when family members were diagnosed, especially if they developed heart disease at an early age
Diagnostic confirmation: Specify whether the patient’s condition is genetically confirmed or clinically diagnosed
Treatment response: Note the patient’s cholesterol levels and response to medications, as this impacts ongoing care planning
Associated conditions: Document any related cardiovascular complications or heart disease in the patient or family members
The National Heart, Lung, and Blood Institute offers detailed information on familial hypercholesterolemia diagnosis and treatment that can support clinical decision-making.
Documentation: “Patient has genetically confirmed heterozygous familial hypercholesterolemia with LDL of 250 mg/dL” Correct Code: E78.01
Documentation: “Patient reports father and grandfather both had high cholesterol requiring medication in their 40s” Correct Code: Z83.49
Documentation: “Patient presents with LDL of 300 mg/dL; family history of premature heart disease; evaluation for familial hypercholesterolemia pending” Initial Code: Z83.49 (family history) until diagnosis confirmed, then E78.01
Documentation: “Well visit for cholesterol screening due to strong family history of hyperlipidemia” Correct Code: Z83.49 as secondary diagnosis to justify screening
Billing specialists should understand that proper use of these codes affects:
Insurance authorization: Some insurers require family history codes to approve early screening or genetic testing
Medical necessity: Family history codes support the need for more aggressive treatment or preventive services
Risk adjustment: The active diagnosis code E78.01 impacts risk adjustment scores in value-based care models
Coverage policies: Documentation must support medical necessity for specialty medications or genetic counseling
Using both the active condition code (E78.01) and family history code (Z83.49) when applicable provides the most complete picture and supports comprehensive reimbursement.
Health IT professionals implementing these coding guidelines should ensure EHR systems:
ICD-10 codes are updated annually, though these particular codes have remained stable. Medical coders should:
Accurate use of ICD-10 codes for hyperlipidemia, including E78.01 for diagnosed FH and Z83.42/Z83.49 for family history, supports patient care, evidence-based screening, and proper reimbursement. Clear documentation, ongoing training, and robust EHR systems help healthcare teams identify at-risk patients and improve outcomes for inherited cholesterol disorders.
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