目的:先天性主动脉瓣上狭窄是一种罕见的先天性流出道梗阻,其长期结局几乎没有报道。本研究旨在概述先天性主动脉瓣上狭窄手术修复后的结果。
方法:对已发表的文献进行了系统综述,包括报告考虑>20例患者的儿童或成人在主动脉瓣上狭窄修复术后长期临床结局(>2年)的观察性研究.早期风险,对晚期事件发生率和事件发生时间数据进行汇总,并输入微观模拟模型,以估计30年的结局.预期寿命与年龄相比,性别和出身匹配的一般人群。
结果:包括23种出版物,共纳入1,472例患者(13,125例患者-年;合并平均随访:9.0(6.2)年;中位随访:6.3年).手术修复的平均年龄为4.7(5.8)岁,最常用的手术技术是单补片修复(43.6%)。合并的早期死亡率为4.2%(95%CI:3.2-5.5%),晚期死亡率为0.61%(95%CI:0.45-0.83)/患者年。基于微观模拟,在30年的时间范围内,据估计,普通瓣上主动脉瓣狭窄修复术患者(平均年龄:4.7岁)的平均预期寿命为配对普通人群预期寿命的90.7%(95%可信间期:90.0~91.6%).基于微观模拟的30年心肌梗死风险为6.0%(95%CrI:5.1-6.5)和再干预31.3%(95%CrI:29.6-33.4%),其中27.2%(95%CrI:25.8-29.1)是由于修复功能障碍。
结论:主动脉瓣上狭窄手术修复后,30年生存率低于匹配的普通人群生存率,并且再干预的终生风险相当大。因此,建议对心血管系统,特别是残余狭窄和冠状动脉梗阻进行终身监测.
OBJECTIVE: Congenital supravalvular aortic stenosis (SVAS) is a rare form of congenital outflow tract obstruction and long-term outcomes are scarcely reported. This study aims to provide an overview of outcomes after surgical repair for congenital SVAS.
METHODS: A systematic
review of published literature was conducted, including observational studies reporting long-term clinical outcome (>2 years) after SVAS repair in children or adults considering >20 patients. Early risks, late event rates and time-to-event data were pooled and entered into a microsimulation model to estimate 30-year outcomes. Life expectancy was compared to the age-, sex- and origin-matched general population.
RESULTS: Twenty-three publications were included, encompassing a total of 1472 patients (13 125 patient-years; pooled mean follow-up: 9.0 (6.2) years; median follow-up: 6.3 years). Pooled mean age at surgical repair was 4.7 (5.8) years and the most commonly used surgical technique was the single-patch repair (43.6%). Pooled early mortality was 4.2% (95% confidence interval: 3.2-5.5%) and late mortality was 0.61% (95% CI: 0.45-0.83) per patient-year. Based on microsimulation, over a 30-year time horizon, it was estimated that an average patient with SVAS repair (mean age: 4.7 years) had an observed life expectancy that was 90.7% (95% credible interval: 90.0-91.6%) of expected life expectancy in the matched general population. The microsimulation-based 30-year risk of myocardial infarction was 8.1% (95% credible interval: 7.3-9.9%) and reintervention 31.3% (95% credible interval: 29.6-33.4%), of which 27.2% (95% credible interval: 25.8-29.1) due to repair dysfunction.
CONCLUSIONS: After surgical repair for SVAS, 30-year survival is lower than the matched-general-population survival and the lifetime risk of reintervention is considerable. Therefore, lifelong monitoring of the cardiovascular system and in particular residual stenosis and coronary obstruction is recommended.