ICD-10-CM 2027
ICD-10 code for familial hypercholesterolemia
From the official ICD-10-CM alphabetic index entry “Hypercholesterolemia (essential) (primary) (pure) › familial”. Page updated September 29, 2026.
About coding familial hypercholesterolemia
The ICD-10-CM code for familial hypercholesterolemia is E78.019 (Familial hypercholesterolemia, unspecified). The official index lists 2 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E78.019.
Within E78.01, choose E78.019 (unspecified) only when documentation doesn't support a more specific option: Homozygous familial hypercholesterolemia [HoFH] (E78.010) and Heterozygous familial hypercholesterolemia [HeFH] (E78.011).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific familial hypercholesterolemia codes (official index)
Common questions about familial hypercholesterolemia ICD-10 codes
What is the ICD-10 code for familial hypercholesterolemia?
- E78.019 — Familial hypercholesterolemia, unspecified. More specific codes apply when the documentation supports them: E78.010, E78.011.
What is the ICD-10 code for homozygous [hofh] familial hypercholesterolemia?
- E78.010 — Homozygous familial hypercholesterolemia [HoFH].
What is the ICD-10 code for heterozygous [hefh] familial hypercholesterolemia?
- E78.011 — Heterozygous familial hypercholesterolemia [HeFH].
Is E78.019 billable?
- Yes. E78.019 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.019 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.019 as a principal diagnosis.
Is E78.019 a CC or MCC?
- No. E78.019 is neither a CC nor an MCC under MS-DRG v44.0.