Treatment Treatment Considerations
ÎIndividuals with FH have a very high lifetime risk of CHD and are at very high risk of premature onset CHD.
ÎEarly treatment is highly beneficial. Long-term drug therapy of patients with FH can substantially reduce or remove the excess lifetime risk of CHD due to the genetic disorder and can lower CHD event rates in FH patients to levels similar to those of the general population.
ÎFH requires lifelong treatment and regular follow-up.
ÎBoth children and adults with LDL-C ≥ 190 mg/dL (or non–HDL-C ≥ 220 mg/dL) after lifestyle changes will require drug therapy.
ÎFor adult FH patients (≥ 20 years of age), drug treatment to achieve an LDL-C reduction ≥ 50% should be initiated.
ÎStatins should be the initial treatment for all adults with FH. Risk Factors
ÎRisk factors are the same in FH as in the general population and require aggressive management to reduce CHD risk, with special attention to smoking cessation.
ÎRegular physical activity, a healthy diet and weight control should be emphasized.
ÎBlood pressure should be treated to < 140/90 mm Hg (or < 130/80 mm Hg in those with diabetes).
ÎLow-dose aspirin (75-81 mg per day) should be considered in those at high CHD or stroke risk.
ÎRisk stratification algorithms should NOT be used.
Comment: Individuals with FH are at high CHD risk. CHD risk in the FH patient is not adequately predicted by any conventional risk assessment tools. Therefore, assessment of 10-year risk is NOT recommended
ÎAll FH patients require lifestyle management as well as lipid-lowering drug therapy.
ÎConsider referral to a lipid specialist with expertise in FH if LDL-C concentrations are not reduced by ≥ 50% or if patients are at high risk.
Lifestyle Modifications
ÎPatients with FH should be counseled regarding the following lifestyle modifications:
• Reduced intakes of saturated fats and cholesterol: total fat 25-35% of energy intake, saturated fatty acids < 7% of energy intake, dietary cholesterol < 200 mg/d.
• Use of plant stanol or sterol esters 2 g/d. • Use of soluble fiber 10-20 g/d. • Physical activity and caloric intake to achieve and maintain a healthy body weight. • Limitation of alcohol consumption. • Emphatic recommendation to avoid use of tobacco product.
ÎClinicians are encouraged to refer patients to registered dietitians or other qualified nutritionists for medical nutrition therapy.
