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Hypercholesterolaemia - familial - Management
How do I specifically manage high cholesterol in an adult with heterozygous familial hypercholesterolaemia?

  • Do not use cardiovascular disease risk assessment tools to guide management, because people with familial hypercholesterolaemia (FH) are already at a high risk of premature coronary heart disease.
  • Adults with confirmed heterozygous FH at particularly high risk of a coronary event should be managed by a specialist with expertise in FH.
  • Prescribe a high intensity statin to achieve a target reduction in low-density lipoprotein cholesterol (LDL-C) concentration of greater than 50% from baseline:
    • Start simvastatin 40 mg, atorvastatin 20 mg, or rosuvastatin 10 mg once daily (each preferably to be taken at night, especially simvastatin):
    • Recheck serum lipids at 4–12 weeks. If the target has not been reached:
      • If taking simvastatin 40 mg, either increase to simvastatin 80 mg daily or, if there are particular concerns about the risk of myopathy with simvastatin 80 mg, change to atorvastatin 40 mg or rosuvastatin 20 mg daily.
      • If simvastatin 80 mg fails to achieve the target, change to atorvastatin 80 mg or rosuvastatin 40 mg daily.
      • If taking atorvastatin 20 mg or rosuvastatin 10 mg, titrate up to the maximum licensed or tolerated dose to achieve the target.
    • See also Special considerations in women and girls.
  • If statins are not tolerated due to new-onset muscle pain (often associated with increased levels of muscle enzymes such as creatine kinase), significant gastrointestinal disturbance, or alterations in liver function tests (LFTs):
  • If statins are contraindicated or not tolerated:
    • Refer to a specialist with expertise in FH.
    • Provided creatine kinase and LFTs have returned to normal, consider prescribing ezetimibe 10 mg once daily instead of a statin whilst the person is waiting to be seen by the specialist.
  • If statin monotherapy fails to achieve the target:
    • Prescribe ezetimibe 10 mg once daily in addition to a statin.
    • If ezetimibe is contraindicated or not tolerated, refer to a specialist with expertise in FH (for consideration for treatment with a bile acid sequestrant [resin], nicotinic acid, or a fibrate).
  • If combined treatment with a statin and ezetimibe fails to achieve the target:
    • Refer to a specialist with expertise in FH.

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