• Gen Thorac Cardiovasc Surg · Aug 2011

    Case Reports

    Patch annulo-aortoplasty in an adult patient with congenital supravalvular aortic stenosis and a small aortic annulus.

    • Naoto Morimoto, Keisuke Morimoto, Yoshihisa Morimoto, Akiko Tanaka, Toshihito Sakamoto, Kenji Okada, and Yutaka Okita.
    • Department of Cardiovascular Surgery, Himeji Cardiovascular Center, 520 Saisho-ko, Himeji, Hyogo, Japan. naotofrcs@gmail.com
    • Gen Thorac Cardiovasc Surg. 2011 Aug 1;59(8):569-71.

    AbstractA 39-year-old woman with familial homozygous hypercholesterolemia had supravalvular and valvular aortic stenosis. Modified Nick's procedure and aortic valve replacement was performed to relieve both the supravalvular and annular stenoses. At surgery, the ascending aorta was found to be narrowing at the level of the sinotubular junction, which was compatible with congenital supravalvular aortic stenosis. Histological examination of the aortic cusps showed sclerotic change due to hypercholesterolemia. These findings indicated that familial homozygous hypercholesterolemia caused valvular aortic stenosis and exacerbated congenital supravalvular aortic stenosis.

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