Ross-Konno procedure and ostial plasty in a child with homozygous hypercholesterolemia: case report.

Although the Ross procedure is preferred for aortic valve replacement in young and female patients, there are no reported cases of hypercholesterolemic aortic pathology due to homozygous familial hypercholesterolemia. Long-term durability of the pulmonary autograft in patients with postoperative high blood cholesterol levels is of interest. A 14-year-old girl with homozygous familial hypercholesterolemia who underwent the Ross-Konno procedure and left coronary artery ostial plasty was followed for 57 months, with pulmonary autograft function, coronary arteries and lipid profile being monitored. There were no signs of narrowing, insufficiency or calcification of the pulmonary autograft; neither was there any narrowing in the left main coronary ostium. The patient's total cholesterol level was reduced from 897 to 262 mg/dl by use of anti-lipidemic medication and weekly lipid apheresis. Follow up data suggest that a pulmonary autograft may be preferable in children with hypercholesterolemic aortic valvular pathology, as well as in children with aortic valvular diseases of other etiologies.