Why the study?
Homozygous familial hypercholesterolemia frequently causes complex aortic root stenosis with a small annulus, but surgical reports are limited to mechanical valve or root replacement and annular enlargement.
Demonstrates the feasibility of using a cryopreserved homograft with a hemi Cabrol technique for complex aortic root stenosis in homozygous familial hypercholesterolemia.
May be an option in select HoFH patients; hypothesis-generating and should not yet change practice.
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.
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Pagni et al. (2026) studied this question.
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