A 28-year-old female presented with 12 months of progressive dyspnea and 6 months of exertional angina, now limiting daily activities. On examination (Fig. 1; Panel A), she had xanthelasma, arcus senilis, and a grade 4/6 ejection systolic murmur. Lab investigations revealed severe dyslipidemia {low-density lipoprotein cholesterol (LDL-c) −503mg/dl (13mmol/L), Triglycerides-90.0 mg/dl (1.0mmol/L), high-density lipoprotein-51.0 mg/dl (1.3mmol/L)}. Echocardiography showed severe supravalvular aortic stenosis (SVAS) with moderate aortic regurgitation (Fig.
