Diagnosis: Hyperlipidemia
Treatment: Repatha
The insurer denied: Repatha
The denial is: Overturned

The patient is a female with a history of hyperlipidemia, dilated ascending aorta and coronary artery calcification. Her coronary artery calcium score was 223, placing her in the 95th percentile for her age, sex and race. On atorvastatin 40 milligrams (mg) daily, her low density lipoprotein (LDL) cholesterol level was 170 milligrams per deciliter (mg/dl). Her atorvastatin dose was increased to 80 mg daily and ezetimibe was started. The atorvastatin was later discontinued due to myalgias. Subsequently her LDL cholesterol level was 243 mg/dl. The medical necessity for Repatha is the subject under review.

Based on the documentation provided Repatha is medically necessary in this instance. This patient has marked hyperlipidemia with an LDL cholesterol level (LDL-C) of 243 mg/dl while on treatment with ezetimibe. On high intensity atorvastatin (40 mg daily, her maximally tolerated dose) her level was 170 mg/dl, which was still well above her recommended goal level of less than 70 mg/dl, given her known coronary atherosclerosis. Based on her LDL cholesterol level she meets criteria for a diagnosis of phenotypic heterozygous familial hypercholesterolemia.

In addition to this patient's phenotypic heterozygous familial hypercholesterolemia, her coronary artery calcium score is indicative of significant coronary artery atherosclerosis. Per a panel of experts convened by the American Society for Preventive Cardiology "Clinical atherosclerotic cardiovascular disease (ASCVD) includes acute coronary syndromes, history of myocardial infarction (MI), stable or unstable angina, coronary or other arterial revascularization, stroke, transient ischemic attack (TIA), or peripheral arterial disease presumed to be of atherosclerotic origin, as well as other forms of atherosclerotic vascular disease including significant atherosclerosis of the coronary, carotid, iliofemoral circulations, and the aorta." This patient meets this definition. Current guidelines endorse the use of Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors for patients with clinical ASCVD whose LDL cholesterol levels remain above goal despite maximally tolerated statin therapy.