The American College of Cardiology and the American Heart Association guidelines recommend treatment of patients with severe hypercholesterolemia (low-density lipoprotein cholesterol [LDL-C] ≥190 mg/100 ml) with a high-intensity statin. However, atherosclerotic cardiovascular disease (ASCVD) risk, even among those with severe hypercholesterolemia, is heterogeneous, and coronary artery calcium (CAC) scoring may be used to clarify risk. We sought to evaluate CAC in patients with severe hypercholesterolemia and measure its impact on real-world statin prescriptions. We identified patients with at least 1 LDL-C ≥190 mg100 ml who had a CAC scoring in the Community Benefit of No-Charge Calcium Score Screening Program (CLARIFY) study (NCT04075162) between 2014 and 2020. We explored the CAC distribution, factors associated with CAC >0, and ASCVD risk (myocardial infarction, stroke, revascularization, death). A total of 1,904 patients (1.257 women, aged 57.8 ± 9.3 years) with severe hypercholesterolemia were included. LDL-C ranged from 190 to 524 mg100 ml (mean 215.5 ± 27 mg100 ml). A total of 864 patients (45.4%) had CAC = 0 and 1,561 (82%) had CAC <100. In patients with LDL-C ≥250 mg100 ml, 67 (36.6%) had CAC = 0. Age, male gender, smoking, diabetes, systolic blood pressure, and obesity (ps ≤0.001) were associated with CAC >0. In patients with LDL-C ≥190 mg100 ml, CAC was associated with a higher risk for ASCVD events (CAC ≥100 vs CAC <100, hazard ratio 3.57 [1.81 to 7.04], p <0.001). A higher CAC category was associated with increased statin use after CAC scoring (p <0.001). In patients with severe hypercholesterolemia, 45% had CAC = 0, which was associated with a significantly lower ASCVD risk. CAC was associated with statin prescription and cholesterol lowering. In conclusion, CAC scoring may be used to clarify ASCVD risk in this heterogeneous population with severe hypercholesterolemia.
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