Tedoriya T, Kawasuji M, Sakakibara N, Hirano K, Watanabe Y, Iwa T
Department of Surgery (1), Kanazawa University School of Medicine, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1991 Nov;39(11):2092-5.
Binovular twins with congenital supravalvular aortic stenosis underwent Doty's extended aortoplasty. Neither twins displayed signs of Williams syndrome. Peak systolic pressure gradients of 65 and 70 mmHg, respectively, were measured across the stenotic portion at the ascending aortae. After surgery, the pressure gradients were reduced to 15 and 20 mmHg, respectively, at the aortic valves. No new aortic regurgitation was observed postoperatively. It is important that the ascending aorta, including the stenotic part, is symmetrically incised into the noncoronary and the right coronary sinus of Valsalva. The fibrous ring should be removed as completely as possible. Doty's extended aortoplasty is a safe and effective procedure for supravalvular aortic stenosis.
患有先天性主动脉瓣上狭窄的双卵双胎接受了多蒂（Doty）扩大主动脉成形术。这对双胞胎均未表现出威廉姆斯综合征的体征。在升主动脉狭窄部位测得的收缩期峰值压力梯度分别为65和70 mmHg。术后，主动脉瓣处的压力梯度分别降至15和20 mmHg。术后未观察到新的主动脉瓣反流。重要的是，包括狭窄部分在内的升主动脉要对称地切入瓦尔萨尔瓦（Valsalva）无冠状动脉窦和右冠状动脉窦。应尽可能完全切除纤维环。多蒂扩大主动脉成形术是治疗主动脉瓣上狭窄的一种安全有效的手术方法。
