Familial Hypercholesterolemia: The Difficult to Manage Patient

A 60-year-old asymptomatic, diabetic, overweight woman with Familial Hypercholesterolemia (FH) has been under my care for one year. LDL-C levels have ranged between 250 and 300 mg/dL. She has had side effects (myalgias, headaches, and fatigue) from six of the seven statins. Vitamin D has been repleted and CoQ10 has been tried. Dietary changes, weight loss, and daily exercise have had an inconsequential impact on her lipids.

Past Medical History:
  1. Small Inferior Myocardial Infarction at age 50.
  2. Coronary artery bypass grafting at age 53.
  3. Stents to the saphenous vein graft to the right coronary artery and native left circumflex coronary artery at age 57 and 59 respectively.
  4. Hypothyroidism
  5. Hypertension
  6. Non-valvular Paroxysmal Atrial Fibrillation
  7. ECHO documentation of moderate valvular and mild supravalvular Aortic Stenosis1
  1. Rivaroxaban
  2. Levothyroxine
  3. ASA
  4. Metoprolol
  5. Ramapril
  6. Vitamin D
  7. Eicosapentaenoic acid and docosahexaenoic acid
Physical Exam:
  1. BP 125/78 mm Hg
  2. HR 58 BPM
  3. BMI 30.02 Kg/m2
  4. Waist circumference at the top of the iliac crest at end-expiration 37"
  5. 2/6 crescendo-decrescendo systolic murmur
  6. Bilateral carotid bruits
  7. Intact peripheral pulses
  8. Corneal arcus (present by history since age 20)
  9. Small bilateral Achilles tendon xanthomas
  1. Total cholesterol 350 mg/dL
  2. LDL-C 265 mg/dL
  3. HDL-C 45 mg/dL
  4. TG 200 mg/dL

ECG: Normal sinus rhythm with old IMI. Borderline LVH.

What are her current treatment options?

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