9
4.2. Severe Hypercholesterolemia (LDL-C ≥190 mg/dL
[≥4.9 mmol/L])
COR LOE
Recommendations
I B-R 1. In patients 20 to 75 years of age with an LDL-C level of 190
mg/dL (≥4.9 mmol/L) or higher, maximally tolerated statin
therapy is recommended.
IIa B-R 2. In patients 20 to 75 years of age with an LDL-C level of 190
mg/dL (≥4.9 mmol/L) or higher who achieve less than a 50%
reduction in LDL-C while receiving maximally tolerated
statin therapy and/or have an LDL-C level of 100 mg/dL
(≥2.6 mmol/L) or higher, ezetimibe therapy is reasonable.
IIb B-R 3. In patients 20 to 75 years of age with a baseline LDL-C level
≥190 mg/dL (≥4.9 mmol/L), who achieve less than a 50%
reduction in LDL-C levels and have fasting triglycerides
≤300 mg/dL (≤3.4 mmol/L) while taking maximally
tolerated statin and ezetimibe therapy, the addition of a bile
acid sequestrant may be considered.
IIb B-R 4. In patients 30 to 75 years of age with heterozygous FH
and with an LDL-C level of 100 mg/dL (≥2.6 mmol/L)
or higher while taking maximally tolerated statin and
ezetimibe therapy, the addition of a PCSK9 inhibitor may be
considered.
IIb C-LD 5. In patients 40 to 75 years of age with a baseline LDL-C level
of 220 mg/dL (≥5.7 mmol/L) or higher and who achieve an
on-treatment LDL-C level of 130 mg/dL (≥3.4 mmol/L)
or higher while receiving maximally tolerated statin and
ezetimibe therapy, the addition of a PCSK9 inhibitor may be
considered.
Value Statement:
Uncertain Value
(B-NR)
6. Among patients with FH without evidence of clinical
ASCVD taking maximally tolerated statin and ezetimibe
therapy, PCSK9 inhibitors provide uncertain value at mid-
2018 U.S. list prices.
