Table 8 Clinical recommendations on the treatment of familial hypercholesterolaemia during pregnancy
Clinical recommendations | Class | Level |
|---|---|---|
1. All women with FH who are of child-bearing age, including adolescents, should be educated about the risks of pregnancy; advice on safer and preferred methods of contraception, with minimal cardiovascular risk, and the importance of contraception should be reinforced to prevent unplanned pregnancy | 1 | B |
2. Reinforcement and optimization of heart-healthy behaviours, including diet, physical activity and psychological well-being, should be prioritized before, during and after pregnancy and breastfeeding | 1 | B |
3. Pre-pregnancy counselling should be offered to all women before starting a statin, ezetimibe, PCSK9 inhibitor or other lipid-modifying therapies, and this advice should be reinforced as clinically indicated | 1 | B |
4. Assessment of ASCVD using imaging (for example, CT angiography for coronary artery disease or echocardiography for aortic stenosis) should be offered to women with HoFH or high-risk HeFH before a planned pregnancy | 1 | B |
5. Given that LDL-cholesterol and triglyceride concentrations increase during pregnancy, assessment of plasma lipids and lipoprotein levels should not routinely be considered, unless the results will be used to change management, as in women with HoFH | 2 | B |
6. Bile acid sequestrants should be considered to treat hypercholesterolaemia, ideally 3 months before a planned pregnancy, as well as during pregnancy and lactation; routine monitoring for malabsorption of fat-soluble vitamins (particularly vitamin K with an international normalized ratio) and folate should also be considered | 2 | B |
7. Statins and other systemically absorbed cholesterol-lowering drugs should ideally be discontinued 3 months before planned conception and during pregnancy and lactation. If a woman with FH becomes pregnant while taking a statin, ezetimibe, a PCSK9 inhibitor or other lipid-modifying therapies, this treatment should be stopped, and she should be reassured that this therapy is unlikely to harm the fetus | 1 | B |
8. In women with HoFH and clinical ASCVD, the continued use of statin therapy should be considered; use of statins, ezetimibe, PCSK9 monoclonal antibodies or other lipid-modifying therapies should particularly be considered after the first trimester, especially if the LDL-cholesterol goal is not achieved and lipoprotein apheresis is not available or feasible | 2 | B |
9. Lipoprotein apheresis should be continued or initiated during pregnancy in women with HoFH, especially in those with established ASCVD and in whom LDL-cholesterol levels are not at guideline-recommended goal; similar advice applies to women with severe HeFH, including those with a lipoprotein(a) concentration ≥ 125 nmol/l (≥60 mg/dl) | 1 | B |
