Surgical Correction for Congenital Valvular and Supravalvular Aortic Stenosis Associated with Coronary Ostial Stenosis in a Child

DOI 1 Citations 10 References
  • Sato Masataka
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Hiramatsu Yuji
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Kato Hideyuki
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Matsubara Muneaki
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Tokunaga Chiho
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Kanemoto Shinya
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Noma Mio
    Department of Cardiovascular Surgery, Tsukuba University Hospital
  • Abe Masakazu
    Department of Cardiovascular Surgery, Ibaraki Children's Hospital
  • Sakakibara Yuzuru
    Department of Cardiovascular Surgery, Tsukuba University Hospital

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  • 左冠動脈入口部狭窄を伴う大動脈弁および弁上狭窄症の幼児に対する手術経験

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Abstract

Supravalvular aortic stenosis is a rare obstructive lesion of the left ventricular outflow tract localized at the level of sinotubular junction. It has been recognized that supravalvular stenosis may occur as a part of Williams syndrome and is sometimes complicated by obstruction of the left main coronary artery. We successfully performed single patch augmentation for supravalvular aortic stenosis and left coronary ostial stenosis with concomitant aortic valvotomy in a child without Williams syndrome. The patient had been followed as congenital bicuspid aortic valvular and supravalvular stenosis. At the age of 3 years, cardiac catheterization revealed an increased pressure gradient of 90mmHg at the left ventricular outflow and newly developed ostial stenosis of the left coronary artery. An oblique incision on the ascending aorta was made above the sinotubular junction and extended leftward onto the left main coronary artery, and this incision opened the fibrous ridge at the left coronary artery. After commissurotomy for the bicuspid valve, both the supravalvular and ostial stenosis were augmented with a single autologous pericardial patch treated by glutaraldehyde. The pressure gradient was significantly reduced and the ischemic left ventricular dysfunction was eliminated.

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