“… - Patients with FH should be counselled on lifestyle modifications and non‐cholesterol risk factors treated 3,5,17,18,20 ( CoR Strong; LoE Moderate ).
- Therapy should initially aim for at least a 50% reduction in LDL‐cholesterol 1,3,17,18 ( CoR Strong; LoE Moderate ), after which the following therapeutic targets should be considered 3,17,18,21 ( CoR Moderate; LoE Moderate ):
- LDL‐cholesterol <2.5 mmol/L (absence of ASCVD or other major ASCVD risk factors);
- LDL‐cholesterol <1.8 mmol/L (imaging evidence of ASCVD alone or other major ASCVD risk factors); or
- LDL‐cholesterol <1.4 mmol/L (presence of clinical ASCVD).
- Diet and maximally tolerated high potency statins with or without ezetimibe should initially be employed to achieve the above targets 1,3,21,22 ( CoR Strong; LoE High ).
- A PCSK9 inhibitor should be used if targets are not achieved with maximally tolerated statins, ezetimibe and diet 1,3,17,21 ( CoR Strong; LoE High ).
- Patients with FH should continue cholesterol‐lowering therapies during acute illness, such as respiratory infections, unless specifically contra‐indicated 3 ( CoR Strong; LoE Low ).
- Plasma hepatic aminotransferases, creatine kinase, glucose and creatinine should be measured before starting and dose‐titrating statins; creatine kinase should be measured if myalgia is reported; glucose should be monitored with risk of diabetes (checks also apply to children and adolescents) 1,3,5 ( CoR Strong; LoE Moderate ).
- All women of child‐bearing age should be offered pre‐pregnancy counselling, with advice on contraception, before starting a statin and this should be reinforced annually (applies also to adolescent girls) 1,3,5 ( CoR Strong; LoE Moderate ).
- Statins and other systemically absorbed cholesterol‐lowering drugs should be discontinued 3 months before conception, as well as during pregnancy and breastfeeding 1,3,5 ( CoR Strong; LoE Moderate ).
- In asymptomatic patients with heFH, carotid ultrasonography and computed tomography coronary angiography may be used for monitoring the efficacy of cholesterol‐lowering therapy 1,3,12 ( CoR Weak; LoE Moderate ).
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