2027 ICD-10-CM Diagnosis Code E78.010Homozygous familial hypercholesterolemia [HoFH]
ICD-10-CM Codes›E00–E89›E70-E88›E78
- Billable — Valid for Submission
- Chronic Condition
E78.010 is a billable ICD-10-CM diagnosis code for homozygous familial hypercholesterolemia [HoFH]. 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 familial hypercholesterolemia. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Disorders of lipid metabolism.
E78.010 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
Code Classification
Medicare Risk Adjustment (HCC)Billing
E78.010 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.
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.
- Familial hypercholesterolemia
- Familial hypercholesterolemia - homozygous
- Familial hypercholesterolemia due to homozygous LDL receptor mutation
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E78.010: 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.
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.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- 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 E78.010, its category, or a range that includes it.
Excludes2 2
These codes carry an Excludes2 note naming E78.010: 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 classifieddisorder of lipoprotein metabolism (E78.-) names E78.-, which includes this code
- Block R70-R79 Abnormal findings on examination of blood, without diagnosisabnormalities 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.
Clinical InformationClinical
Homozygous Familial Hypercholesterolemia
a rare inherited genetic disorder, one form of hyperlipoproteinemia type ii, characterized by high level of low-density lipoprotein (ldl) which if not treated could elevate the chance of heart attack at an early age.
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 E78.010 replaces the following previously assigned code(s):
- E78.01 - Familial hypercholesterolemia
Questions About E78.010Overview
What is the ICD-10 code for homozygous familial hypercholesterolemia [HoFH]?
The ICD-10-CM code for homozygous familial hypercholesterolemia [HoFH] is E78.010 (sometimes written as E78010). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E78.010 (Familial hypercholesterolemia) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report homozygous familial hypercholesterolemia [HoFH] on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E78.010 group to?
When homozygous familial hypercholesterolemia [HoFH] 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 E78.010 be reported with a code from E34.5?
Not as a rule. The Excludes1 note at block E70-E88, which applies to E78.010, 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 E78.010 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage: E78.010 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).
