Familial Hypercholesterolemia Follow-up
- Author: Mose July, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP more...
Identifying relatives who are carriers of the FH gene allows medical intervention to prevent patients from developing CAD.
In addition to treating hypercholesterolemia, cardiovascular risk factors should be identified and treated aggressively. Advise patients to begin aerobic exercise and, if indicated, a weight-loss program.
The adverse effects of medications used to treat hypercholesterolemia can pose major, though uncommon, complications.
Statin therapy carries a negligible risk of liver toxicity.
Myositis progressing to rhabdomyolysis is a rare but life-threatening complication of statin therapy.
Statins in combination with a variety of medications (particularly cyclosporine, as well as gemfibrozil, verapamil, amiodarone, etc) increase the risk of myositis (see Medication).
Niacin may cause gout, peptic ulcer disease, increased insulin resistance, and severe hepatotoxicity. Fulminant hepatic failure has been reported with time-release niacin therapy.
Prognosis depends heavily on the extent to which LDLc levels can be reduced.
Patients with homozygous FH have and extremely limited life expectancy without major medical intervention.
Treatment of other modifiable risk factors such as smoking, hypertension, and diabetes further decreases the risk of CAD.
Because long-term prospective studies on subjects with FH are not available, precise predictions regarding improved outcomes are difficult.
Adult patients with FH must understand their high risk for premature CAD. Emphasizing the importance of complying with dietary and drug management of their hypercholesterolemia must be emphasized.
Other modifiable risk factors should be identified, and their additive impact on the risk of a cardiovascular event should be explained. Offer assistance with stopping smoking. Explain the importance of exercise and appropriate weight reduction in terms of the lipid and cardiovascular effects and the prevention or improvement in diabetes and hypertension.
For excellent patient education resources, visit eMedicineHealth's Cholesterol Center. Also, see eMedicineHealth's patient education articles High Cholesterol, Cholesterol Charts, Lifestyle Cholesterol Management, Cholesterol-Lowering Medications, and Statins for Cholesterol.
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LDLc Target level,
LDLc level Indicating TLC,
LDLc level for Considering Drug Therapy,
CHD or CHD risk equivalent
(10-y risk >20%)
Optional goal <70
|Moderately high risk:|
More than 2 risk factors
(10-y risk 10-20%)
Optional goal <100
(100-129 may consider drug options)
More than 2 risk factors
(10-y risk 10%)
0-1 risk factor
(160-189 LDL-lowering drug optional)
|*The 2004 update recommended that when statin therapy is initiated in patients at high or moderately high risk, a dose and strength should be chosen that achieves at least a 30-40% LDLc reduction (see Table 3).|
Typical US 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|
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|
|80 mg at bedtime||35-38%||80 mg at bedtime|
|Lovastatin||20-80 mg at supper||25-48%||40 mg at dinner|
|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|
|50-60%||10/20 mg at bedtime|