ICD-10 Code E78.011 – Heterozygous familial hypercholesterolemia [HeFH] (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E78.011 – Heterozygous familial hypercholesterolemia [HeFH]
What it is
E78.011 identifies heterozygous familial hypercholesterolemia, an inherited disorder that causes very high LDL cholesterol from birth. Use it when documentation specifically states HeFH or heterozygous familial hypercholesterolemia.
Clinical signs
Common findings include markedly elevated LDL cholesterol, premature atherosclerotic cardiovascular disease, and a family history of early heart disease. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents HeFH as the diagnosed condition, whether newly identified or longstanding. It may appear in genetics, lipid clinic, cardiology, or primary care notes. Code it only when the record clearly supports heterozygous familial hypercholesterolemia, not general hyperlipidemia.
Do not use for
Do not use E78.011 for unspecified high cholesterol, mixed dyslipidemia, or purely acquired hyperlipidemia. If the chart does not confirm HeFH, check documentation.
Coding tip
Look for exact wording such as “heterozygous familial hypercholesterolemia” or “HeFH” before assigning E78.011.