2026 ICD-10-CM Diagnosis Code E78.011Heterozygous familial hypercholesterolemia [HeFH]

ICD-10-CM Codes›E00–E89›E70-E88›E78

ICD-10-CM E78.011
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E78.011 is a billable ICD-10-CM diagnosis code for heterozygous familial hypercholesterolemia [HeFH]. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as combined heterozygous low density lipoprotein receptor co-occurrent with low density lipoprotein receptor adaptor protein 1 mutations.

Code Identity

ICD-10-CM Code
E78.011
Billable Status
Yes — Valid for Submission
Code Describes
Heterozygous familial hypercholesterolemia [HeFH]
Short Description
Heterozygous familial hypercholesterolemia [HeFH]
Same as the full description in the CMS dataset.
Parent Code
E78.01
Familial hypercholesterolemia

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE78Disorders of lipoprotein metabolism and other lipidemias
This CodeE78.011Heterozygous familial hypercholesterolemia [HeFH]

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Combined heterozygous low density lipoprotein receptor co-occurrent with low density lipoprotein receptor adaptor protein 1 mutations
  • Double heterozygous familial hypercholesterolemia
  • Familial hypercholesterolemia
  • Familial hypercholesterolemia - heterozygous
  • Familial hypercholesterolemia due to heterozygous LDL receptor mutation
  • Low density lipoprotein receptor adaptor protein 1 mutation
  • Low density lipoprotein receptor mutation

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Cholesterolemia(essential) (pure)
      • familial
        • heterozygous
    • HeFH(heterozygous familial hypercholesterolemia)
    • Hypercholesterolemia(essential) (primary) (pure)
      • familial
        • heterozygous [HeFH]

Code History & ChangesHistory

New Code E78.011 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.

Replacement E78.011 replaces the following previously assigned code(s):

  • E78.01 - Familial hypercholesterolemia
FY 2026AddedAdded to the ICD-10-CM code setEffective October 1, 2025.
FY 2026CurrentRevised in the current code setEffective October 1, 2025 through September 30, 2026.

Questions About E78.011Overview

Is E78.011 (Familial hypercholesterolemia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report heterozygous familial hypercholesterolemia [HeFH] on HIPAA-covered claims from October 1, 2025 through September 30, 2026.