Cryopreserved aortic homograft root replacement for supravalvular root stenosis in familial homozygous hypercholesterolemia

Homozygous familial hypercholesterolemia is a genetic disorder resulting in severe elevations of the total cholesterol that is frequently associated with complex aortic root stenosis and small aortic valve annulus. The few reported surgical approaches included variations of mechanical valve/root replacement and annular enlargement. We report a rare case of supravalvular aortic stenosis (SVS) with severe atherosclerotic root dystrophic stenosis with coronary and valve involvement treated with a cryopreserved homograft using a hemi Cabrol technique.