Abstract：
Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus on the optimal approach. This study aimed to analyze the outcomes of surgical treatment of SVAS and determine the optimal strategy. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for residual aortic stenosis and residual stenosis-related reintervention. From December 2008 to December 2023, 98 patients with SVAS undergoing surgical repair in our institution were included [McGoon group, n = 62; Doty group, n = 36]. There were 2 in-hospital deaths and 1 late death. The survival rates at 1, 5, and 15 years were 98.0%, 96.7%, and 96.7%, respectively in the whole cohort. Residual aortic stenosis occurred in 18 patients. Multivariable analysis showed that preoperative gradient ≥ 90 mmHg (P = 0.002) and Williams syndrome (P = 0.002) were incremental risk factors for residual aortic stenosis, but surgical technique (P = 0.579) was not a risk factor for residual aortic stenosis. In the McGoon group, patients with diffuse type had worse freedom from residual aortic stenosis than patients with discrete type (P = 0.007). However, in the Doty group, patients with diffuse type had comparable freedom from residual aortic stenosis to patients with discrete type (P = 0.911). Residual stenosis-related reintervention occurred in 15 patients. Fifteen patients all underwent residual aortic stenosis-related reintervention. Of 15 patients, 6 patients also underwent residual pulmonary stenosis-related reintervention. On multivariate analysis, Williams syndrome (P < 0.001), preoperative sinotubular junction (STJ) z-score < - 3.5 (P = 0.051), and Doty repair (P = 0.033) were found to be independent risk factors associated with residual stenosis-related reintervention. In the whole cohort, freedom from residual stenosis-related reintervention at 1, 5, and 15 years were 97.8%, 89.3% and 76.1%, respectively. Surgical repair of SVAS can be safely achieved using different techniques, with similar long-term mortality. Compared with McGoon repair, Doty repair was significantly associated with decreased residual aortic stenosis rates in patients with diffuse-type SVAS. Patients with preoperative gradient ≥ 90 mmHg or Williams syndrome are more prone to residual aortic stenosis. Surgical technique was not associated with residual aortic stenosis rates. Williams syndrome, preoperative STJ z-score < - 3.5, and Doty repair are associated with higher residual stenosis-related reintervention rates.
摘要：
已经引入了各种外科技术来治疗瓣上主动脉瓣狭窄(SVAS)。然而,关于最优方法没有共识。本研究旨在分析手术治疗SVAS的结果并确定最佳策略。Kaplan-Meier曲线用于证明生存估计。Cox比例风险模型用于确定残余主动脉瓣狭窄和残余狭窄相关再干预的危险因素。从2008年12月到2023年12月，我们机构接受手术修复的98例SVAS患者被纳入[McGoon组，n=62；Doty组，n=36]。有2例住院死亡和1例晚期死亡。1、5、15年生存率为98.0%,96.7%,96.7%,分别在整个队列中。18例患者发生残余主动脉瓣狭窄。多因素分析显示术前梯度≥90mmHg(P=0.002)和Williams综合征(P=0.002)是主动脉瓣狭窄的增量危险因素,但手术技术(P=0.579)不是主动脉瓣残余狭窄的危险因素.在麦戈恩集团，与离散型患者相比,弥漫型患者的残余主动脉瓣狭窄发生率较差(P=0.007).然而,在多蒂小组中，与离散型患者相比,弥漫型患者无残余主动脉瓣狭窄(P=0.911).15例患者发生残余狭窄相关再介入。15例患者均接受了残余主动脉瓣狭窄相关的再干预。15名患者中,6例患者还接受了残余肺动脉狭窄相关的再干预。在多变量分析中，威廉姆斯综合征（P<0.001），术前窦管连接（STJ）z评分<-3.5（P=0.051），发现Doty修复（P=0.033）是与残余狭窄相关的再干预相关的独立危险因素。在整个队列中，在1年、5年和15年的残余狭窄相关再介入的自由度为97.8%,89.3%和76.1%，分别。SVAS的手术修复可以使用不同的技术安全地实现，长期死亡率相似。与McGoon修复相比，Doty修复与弥漫型SVAS患者的残余主动脉狭窄率降低显着相关。术前梯度≥90mmHg或Williams综合征的患者更容易发生残余主动脉瓣狭窄。手术技术与残余主动脉狭窄率无关。威廉姆斯综合症,术前STJz评分<-3.5,Doty修复与较高的残余狭窄相关再介入率相关。
