52
Management
4.2.4.4. Severe Hypercholesterolemia With Clinical or Genetic
Confirmation of Homozygous Familial Hypercholesterolemia
(HoFH)
COR LOE
Recommendations
1 B-NR
1. In adults with clinical and/or genetic confirmation of HoFH,
consultation with a lipid specialist is recommended for
consideration of advanced LDL-C–lowering drug therapies
and/or lipoprotein apheresis to lower LDL-C.
1 B-R
2. In adults with clinical or genetic confirmation of HoFH,
treatment with maximally tolerated statin therapy is
recommended to reduce ASCVD risk.
2a B-R
3. In adults with clinical or genetic confirmation of HoFH
currently on maximally tolerated statin therapy, the addition
of ezetimibe, PCSK9 mAb, and/or bempedoic acid is
reasonable to lower LDL-C.
2b B-R
4. In adults with clinical or genetic confirmation of HoFH
currently on maximally tolerated statin therapy, ezetimibe, and
PCSK9 mAb with an LDL-C ≥100 mg/dL (2.6 mmol/L), the
addition of evinacumab may be reasonable to lower LDL-C.
2b C-LD
5. In adults with clinical or genetic confirmation of HoFH
currently on maximally tolerated statin therapy, ezetimibe,
and PCSK9 mAb with LDL-C ≥100 mg/dL (2.6 mmol/L),
the addition of lomitapide with regular monitoring for
hepatic safety may be reasonable to lower LDL-C.
Table 17. Diabetes-Specific Risk Enhancers That Are
Independent of Other Risk Factors in Diabetes
Risk Enhancers
• Long duration (≥10 y for type 2 diabetes or ≥20 y for type 1 diabetes)
• Albuminuria ≥30 µg of albumin/mg creatinine
• eGFR <60 mL/min/1.73 m
2
• Retinopathy
• Neuropathy
• ABI <0.9
ABI indicates ankle-brachial index; eGFR, estimated glomerular filtration rate; and y, years.
Reprinted with permission from Grundy et al. Copyright © 2018 American Heart
Association, Inc. and American College of Cardiolog y Foundation.
