AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2025 Issue 4; New/Revised ICD-10-CM Codes

Familial Hypercholesterolemia

Code E78.01, Familial hypercholesterolemia, was expanded to create new codes to identify Homozygous familial hypercholesterolemia [HoFH] (E78.010), Heterozygous familial hypercholesterolemia [HeFH] (E78.011), and Familial hypercholesterolemia, unspecified (E78.019). Familial hypercholesterolemia is an inherited genetic disorder that interferes with the body’s ability to remove low-density lipoprotein cholesterol (LDL-C), the “bad cholesterol” from the blood. LDL-C causes blockage in the arterial wall and narrows the lumens, which increases the risk of coronary heart disease, acute myocardial infarction, and stroke. There are two types of familial hypercholesterolemia: heterozygous and homozygous. Heterozygous familial hypercholesterolemia (HeFH) occurs...

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