2027 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 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

E78.011 is a billable ICD-10-CM diagnosis code for heterozygous familial hypercholesterolemia [HeFH]. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. Coders also document this condition as combined heterozygous low density lipoprotein receptor co-occurrent with low density lipoprotein receptor adaptor protein 1 mutations. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Disorders of lipid metabolism.

E78.011 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 47 for payment year 2027.

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]

Medicare Risk Adjustment (HCC)Billing

E78.011 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model, but it maps to payment categories in the other CMS models shown below.

CMS-HCC V28 (Medicare Advantage Payment Model)
Not mapped
no payment category in the model that pays 100% of MA risk scores
Prior Model (CMS-HCC V24)
Not mapped under V24
this diagnosis newly risk-adjusts under the V28 model
Part D (RxHCC)
RxHCC 47 — Disorders of Lipoid Metabolism
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

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

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E78.011: its own notes plus those printed at E78, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From E78 Disorders of lipoprotein metabolism and other lipidemias applies to 18 codes
From Block E70-E88 Metabolic disorders applies to 286 codes
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)
From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block E70-E88 Metabolic disorders applies to 286 codes
  • Ehlers-Danlos syndromes (Q79.6-)

Notes

General instructions on how these codes are used.

From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name E78.011, its category, or a range that includes it.

Excludes2 2

These codes carry an Excludes2 note naming E78.011: its condition is not part of those codes, and both may be reported when the patient has both.

  • E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
    disorder of lipoprotein metabolism (E78.-) names E78.-, which includes this code
  • Block R70-R79 Abnormal findings on examination of blood, without diagnosis
    abnormalities of lipids (E78.-) names E78.-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END010
Disorders of lipid metabolism
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Hyperlipoproteinemia Type II

    a group of familial disorders characterized by elevated circulating cholesterol contained in either low-density lipoproteins alone or also in very-low-density lipoproteins (pre-beta lipoproteins).

Patient EducationClinical

Cholesterol

Cholesterol is a waxy, fat-like substance that's found in all the cells in your body. Your body needs some cholesterol to make hormones, vitamin D, and substances that help you digest foods. Your body makes all the cholesterol it needs. Cholesterol is also found in foods from animal sources, such as egg yolks, meat, and cheese.

The full article covers:

  • What is cholesterol?
  • What are HDL, LDL, and VLDL?
  • What causes high cholesterol?
  • What can raise my risk of high cholesterol?
  • What other health problems can high cholesterol cause?
  • How is high cholesterol diagnosed?
  • How can I lower my cholesterol?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Code History & ChangesHistory

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 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About E78.011Overview

What is the ICD-10 code for heterozygous familial hypercholesterolemia [HeFH]?

The ICD-10-CM code for heterozygous familial hypercholesterolemia [HeFH] is E78.011 (sometimes written as E78011). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

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, 2026 through September 30, 2027.

What MS-DRG does E78.011 group to?

When heterozygous familial hypercholesterolemia [HeFH] is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.

Can E78.011 be reported with a code from E34.5?

Not as a rule. The Excludes1 note at block E70-E88, which applies to E78.011, lists "androgen insensitivity syndrome (E34.5-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

Does E78.011 risk-adjust for Medicare Advantage payment?

Not for Medicare Advantage: E78.011 maps to no category in the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 47 (Disorders of Lipoid Metabolism).