Abstract：
This study investigated long-term outcomes and factors associated with reoperations in patients who underwent surgical repair of congenital supravalvar aortic stenosis (SVAS). A total of 39 consecutive patients who underwent congenital SVAS repair from 1999 through 2018 were included. Aortic root geometry was evaluated by measuring the ratio of the sinotubular junction diameter to the aortic annulus diameter (STJ/AVA) on echocardiography and proportion of intercommissural distance (ICD) of each sinus on computed tomography. The median age and weight at the time of operation were 4.3 years and 16.9 kg, respectively. Williams syndrome was associated in 25 patients (64.1%). Modified simple sliding aortoplasty (MSSA) was mostly used (n = 35, 89.7%). The median follow-up duration was 9.5 years. There were no early deaths and 1 late death. Overall survival rate was 97.0% at 15 years. There were 7 reoperations during follow-up. Freedom from reoperation for left ventricular outflow tract obstruction and all-cause reoperation were 91.9% and 80.4%, respectively. Age younger than 2 years at initial repair were associated with all-cause reoperation in the univariable analysis. In 35 patients who underwent MSSA, the degree of aortic regurgitation was equal to or less than mild in all patients during follow-up. Their median STJ/AVA on postoperative echocardiography was 0.95 (0.84-1.02). SVAS repair with MSSA provided excellent long-term survival with well-preserved aortic valve competence. Age younger than 2 years at initial repair might be associated with reoperation.
摘要：
这项研究调查了接受先天性主动脉瓣上狭窄（SVAS）手术修复的患者的长期结局和与再次手术相关的因素。共纳入了从1999年到2018年接受先天性SVAS修复的39例连续患者。通过在超声心动图上测量窦管连接直径与主动脉瓣环直径之比（STJ/AVA）以及在计算机断层扫描上测量每个窦的连合距离（ICD）的比例来评估主动脉根的几何形状。手术时的中位年龄和体重分别为4.3岁和16.9kg，分别。威廉姆斯综合征在25例患者中相关(64.1%)。大多数使用改良的简单滑动主动脉成形术（MSSA）（n=35，89.7％）。中位随访时间为9.5年。没有早期死亡和1晚期死亡。15年总生存率为97.0%。随访期间有7次再次手术。左心室流出道梗阻和全因再手术的发生率分别为91.9%和80.4%,分别。在单变量分析中，初次修复时年龄小于2岁与全因再手术有关。在35例接受MSSA的患者中，在随访期间,所有患者的主动脉瓣反流程度均等于或小于轻度.术后超声心动图的STJ/AVA中位数为0.95（0.84-1.02）。使用MSSA进行SVAS修复可提供出色的长期生存率，并保留了良好的主动脉瓣功能。初次修复时年龄小于2岁可能与再次手术有关。
