Forman M B, Kinsley R H, Du Plessis J P, Dansky R, Milner S, Levin S E
S Afr Med J. 1982 Apr 17;61(16):579-82.
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.
虽然冠状动脉粥样硬化过早发生是纯合子家族性高胆固醇血症公认的并发症，但主动脉根部和瓣膜受累在生前并不常被认识到。本文描述了两例因家族性高胆固醇血症导致的主动脉瓣上和瓣膜狭窄病例。两名患者均成功接受了这些病变的手术矫正。强调了通过主动脉根部强化技术和假体斜向植入来解除左心室流出道梗阻的重要性。
