What guidelines exist for the medical care of patients with familial hypercholesterolemia (FH)?

Updated: Oct 04, 2021
  • Author: Mose July, MD, CCD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Answer

The National Cholesterol Education Program (NCEP) ATPIII defined LDLc goals and cutpoints for therapeutic intervention based on risk for CHD (see Table 2 and Table 3). [6, 7]

The guidelines were updated in 2004 to reflect the findings of several interventional trials demonstrating that coronary event rate was reduced after lowering the LDLc well below 100 mg/dL. [8]

The European Society of Cardiology (ESC) and European Atherosclerosis Society (EAS) have also released guidelines for the management of dyslipidemias, available at the ESC site. [27]

In addition, the American College of Cardiology/American Heart Association (ACC/AHA) released updated blood cholesterol guidelines, in 2018; however, they do not address a specific approach to and management of FH. [28] .

Table 2. Recommended Dietary Intake (Open Table in a new window)

 

Food Category

 

Typical US Diet

 

NCEP Diet

 

Diet for FH

Cholesterol, mg/d

500

< 200

100

Total fat, % energy (calories)

40

25-35

20

Saturated fat, % energy (calories)

14

< 7

< 6

Carbohydrate, % energy (calories)

45

50-60

65

Protein, % energy (calories)

Approximately 15

15

N/A

Table 3. Statin and Statin Combination Approved Doses, Expected LDLc Decrease, and Dose Required for 30-40% LDLc Reduction (Open Table in a new window)

 

Statin

 

FDA-Approved Dose

 

Expected LDLc Decrease

 

Dose Required for 30-40% LDLc Reduction

Atorvastatin

10-80 mg daily

35-60%

10 mg

Fluvastatin

20-40 mg at bedtime

20-30%

40 mg qd/bid

40 mg bid

35%

40 mg bid

Extended-release fluvastatin

(Lescol XL)

80 mg at bedtime

35-38%

80 mg at bedtime

Lovastatin

20-80 mg at supper

25-48%

40 mg at dinner

Extended-release lovastatin

(Altoprev)

20-60 mg at bedtime

25-45%

60 mg at bedtime

Pravastatin

40-80 mg at bedtime

30-40%

40 mg at bedtime

Rosuvastatin

10-40 mg daily

40-60%

5 mg daily

Simvastatin

20-80 mg daily at bedtime

35-50%

20 mg at bedtime

Simvastatin + ezetimibe

(Vytorin)

10/20 mg

10/40 mg

10/80 mg

at bedtime

50-60%

10/20 mg at bedtime


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