Surgical correction of combined supravalvular and valvular aortic stenosis in homozygous familial hypercholesterolaemia.
Although premature coronary atherosclerosis is a well-recognized complication of homozygous familial hypercholesterolaemia, involvement of the aortic root and valve has not been recognized frequently during life. Two cases of supravalvular and valvular aortic stenosis due to familial hypercholesterolaemia are described. Both patients underwent successful surgical correction of these lesions. The importance of relieving left ventricular outflow tract obstruction by a technique of aortic root enhancement and oblique insertion of a prosthesis is stressed.
D001021:Aortic Valve / Q000601:surgery; D001024:Aortic Valve Stenosis / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D004562:Electrocardiography; D006350:Heart Valve Prosthesis; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications* / Q000601:surgery; D008297:Male