Surgical Challenges of Familial Hypercholesterolemia

A 21-year-old patient with familial hypercholesterolemia presented with angina caused by ostial stenosis of the left internal mammary artery and severe calcific aortic stenosis with small aortic root 9 years after coronary revascularization. The ostium of the left internal mammary artery was enlarged using a saphenous vein patch through a left supraclavicular incision, which improved left ventricular function. Successful aortic valve replacement with posterior aortic root enlargement was subsequently performed. The surgical management of this condition is discussed briefly.