Adjuvant Therapeutic Choices in Patients With Familial Hypercholesterolemia

A 37-year-old Caucasian man presents to clinic to establish care. At age 18 years, he was diagnosed with homozygous familial hypercholesterolemia (HoFH) after his father died suddenly of a massive myocardial infarction at age 42 years. Two paternal uncles also died suddenly at ages 39 and 45 years. At that time, his low-density lipoprotein cholesterol (LDL-C) was 530 mg/dL, and he was started on a statin and advised to initiate LDL-C apheresis. He notes that he stopped the statin after seven months because "he did not like the way it made him feel." He also discontinued apheresis because it required a 3.5-hour drive each way to reach an academic medical center and his health insurance only covered approximately 30% of the cost. Despite much encouragement, he rejected the possibility of liver transplantation. He stopped following up with a physician. At age 35 years, he sustained an inferior wall myocardial infarction. On cardiac catheterization, he was found to have diffuse multivessel disease with an 80% obstruction in his left main coronary artery, a 75% obstruction in his proximal circumflex artery, and a completely occluded right coronary artery at approximately the middle portion of the vessel. He underwent a three-vessel coronary artery bypass without complication.

The patient is not currently experiencing any symptoms of myocardial ischemia or heart failure. He does not smoke and is walking for 45 minutes daily. He is on an American Heart Association Heart Healthy diet with particular care focused on avoiding saturated and trans fats. His current medications include rosuvastatin 40 mg po qd, ezetimibe 10 mg po qd, colesevelam 3.75 g po qd, aspirin 81 mg po qd, ramipril 10 mg po qd, and carvedilol 12.5 po qd. Vital signs are normal. A physical examination reveals multiple abnormalities. On the current pharmacologic regimen, the patient's fasting lipid profile shows LDL-C 230 mg/dL, HDL-C 67 mg/dL, and triglycerides 97 mg/dL.

Which of the following are reasonable adjuvant therapeutic choices in this patient?

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