Abstract：
This study reviewed the midterm outcomes of supravalvular aortic stenosis (SVAS) repair and determined the risk factors associated with postoperative aortic or pulmonary stenosis. We retrospectively reviewed 225 patients who underwent surgical correction of SVAS from 2010 to 2019. A total of 178 (79.1%), 44 (19.6%) and 3 (1.3%) patients underwent McGoon, Doty, and Brom repair, respectively. The median age at surgery was 2.2 years (interquartile range, 1.2-4.4). The median follow-up time was 3.7 years (interquartile range, 1.9-5.7). Early and late mortality rates were 3.1% and 1.4%, respectively. The overall 5-year survival rate was 97.9%. Eleven patients received reintervention, including 6 (2.8%) reoperations and 5 (2.3%) balloon dilatations. Higher preoperative pressure gradient at the distal ascending aorta or aortic arch was a risk factor for reintervention (P = 0.04). Rates of mortality and complications were not related to the surgical technique. Eleven patients (5.2%) developed sinotubular junction (STJ) stenosis. Freedom from postoperative distal artery stenosis (DAS) of type II SVAS was significantly lower than that of type I (P < 0.01). Higher preoperative pressure gradient at the STJ (P < 0.01) and concomitant bilateral or peripheral pulmonary artery (PA) stenosis (P < 0.01) were risk factors for postoperative DAS. Postoperative PA stenosis occurred more frequently in patients who received bilateral pulmonary arterioplasty (P < 0.01). Postoperative prognosis of the aortic root after SVAS repair was satisfactory. DAS and PA stenosis were common. The results of bilateral pulmonary arterioplasty were unsatisfactory. The surgical timing and technique for PA stenosis should be carefully considered.
摘要：
本研究回顾了瓣上主动脉瓣狭窄（SVAS）修复的中期结果，并确定了与术后主动脉瓣或肺动脉瓣狭窄相关的危险因素。我们回顾性分析了2010年至2019年接受SVAS手术矫正的225例患者。共178人（79.1%），44例（19.6%）和3例（1.3%）患者接受了McGoon，多蒂,和Brom修复，分别。手术时的中位年龄为2.2岁(四分位距，1.2-4.4)。中位随访时间为3.7年(四分位间距，1.9-5.7)。早期和晚期死亡率分别为3.1%和1.4%,分别。5年生存率为97.9%。11名患者接受了再干预，包括6次（2.8%）再次手术和5次（2.3%）球囊扩张。术前远端升主动脉或主动脉弓压力梯度较高是再次干预的危险因素(P=0.04)。死亡率和并发症与手术技术无关。11例患者（5.2％）发生了窦管交界（STJ）狭窄。II型SVAS术后远端动脉无狭窄（DAS）明显低于I型（P<0.01）。术前STJ处压力梯度较高（P<0.01）和伴有双侧或外周肺动脉（PA）狭窄（P<0.01）是术后DAS的危险因素。双侧肺动脉成形术后PA狭窄发生率较高(P<0.01)。SVAS修复后主动脉根部的术后预后令人满意。DAS和PA狭窄常见。双侧肺动脉成形术的结果不令人满意。应仔细考虑PA狭窄的手术时机和技术。
