Surgery for calcific aortic root stenosis in homozygous familial hypercholesterolemia

A 35-year-old female with homozygous familial hyperlipidemia (IIa) was referred to our hospital for an operation against supravalvular and valvular aortic stenosis. She had been treated with low-density lipoprotein apheresis for 20 years, and total cholesterol ranged between 200 and 400 mg/dl under this treatment. She had undergone percutaneous coronary intervention for ostial stenosis of the right coronary artery three times since the age of 19. Unenhanced three-dimensional computed tomography showed supravalvular stenosis, funnelling and heavily calcified aorta. An operation was performed under deep hypothermic circulatory arrest without aortic cross clamping. After the ascending aorta had been replaced with a one-branched vascular graft, arterial perfusion was resumed. The stenosed ascending aorta was resected at the sinotubular junction. Because the aortic root was still extremely small, the noncoronary sinus and the commissure between left and right coronary cusp were incised, and the aortic root was enlarged with linguiform vascular-graft patches. A 21-mm mechanical valve was implanted. The postoperative course was uneventful.