Yagual-Gutiérrez Eliana B, Freire-Barrezueta Isabel M, Hernandez-Duarte Mercedes C, Calero-Solis Isabel J, Maldonado-González David F, Zhune-Villagran Darwin E
Department of Pediatric Cardiovascular Surgery, Children Hospital Roberto Gilbert Elizalde, Guayaquil, Ecuador.
Department of Pediatric Cardiology, Children Hospital Roberto Gilbert Elizalde, Guayaquil, Ecuador.
Arch Cardiol Mex. 2024 Jul 29;95(2):215-9. doi: 10.24875/ACM.24000089.
Supravalvular aortic stenosis (SVAS) is a congenital malformation associated with Williams-Beuren syndrome. It is characterized by severe stenosis of the sinotubular junction. The smooth muscle layer is thickened due to an alteration of the Elastin gene. The treatment is surgical and there are several techniques to relieve obstruction which range from the single-patch technique to the replacement of all three aortic sinuses with three patches. However, the optimal technique is still unclear. The objective of this article is to carry out a bibliographic review of surgical techniques for SVAS and the description of the application of the Doty technique for a 4-year-old infant at the Roberto Gilbert Elizalde Hospital.
瓣上主动脉狭窄（SVAS）是一种与威廉姆斯-贝伦综合征相关的先天性畸形。其特征为窦管交界严重狭窄。由于弹性蛋白基因改变，平滑肌层增厚。治疗方法为手术治疗，有多种解除梗阻的技术，从单补片技术到用三个补片替换所有三个主动脉窦。然而，最佳技术仍不明确。本文的目的是对SVAS的手术技术进行文献综述，并描述多蒂技术在罗伯托·吉尔伯特·埃利萨尔代医院应用于一名4岁婴儿的情况。
