背景:原发性主动脉瓣上狭窄(SVAS)是一种罕见的先天性心血管疾病,可与Williams-Beuren综合征共存,冠状动脉受累,主动脉缩窄和肺动脉狭窄。SVAS修复可以实现低围手术期死亡率,但长期生存仍不太清楚。我们使用了儿科心脏护理协会,基于美国的儿科心脏手术多中心注册,评估SVAS修复后的长期结果。
方法:我们使用Kaplan-Meier图和Cox比例风险回归来检查与出院后死亡相关的因素。这些包括性,年龄组,体重z分数,共存条件(威廉姆斯-贝伦综合征,冠状动脉受累,缩窄和肺动脉狭窄),外科技术,时代定义为早期(1982-1995)或晚期(1996-2003)。通过与2021年的国家死亡指数(NDI)相匹配来评估生存率。
结果:333例患者符合纳入标准,313(94.0%)存活出院,188(60.1%)具有NDI匹配的标识符。平均随访25.2年(IQR:21.1-29.4),发生17人死亡。SVAS修复出院后30年生存率为88.7%(95CI:82.9-94.8%)。婴儿手术和非Williams-Beuren综合征与30年生存率下降相关。从各种修理中,与所有其他类型相比,2窦有更好的结果,除了3窦技术(无显著差异)。调整后的分析显示,婴儿年龄和修复类型与出院后死亡率相关。
结论:这些数据表明,除了与更弥漫性动脉病变相关的婴儿组外,SVAS修复后的长期结局良好。随着技术的不断发展,未来的研究有必要调查其长期结局.
BACKGROUND: Primary supravalvar aortic stenosis (SVAS) is a rare congenital cardiovascular condition that can coexist with Williams-Beuren syndrome, coronary artery involvement, aortic coarctation, and pulmonary artery stenosis. SVAS repair can be achieved with low perioperative mortality, but long-term survival remains less well understood. We used the Pediatric Cardiac Care Consortium, a multicenter United States-based registry for pediatric cardiac operations, to assess long-term outcomes after SVAS repair.
METHODS: We used Kaplan-Meier plots and Cox proportional hazards regression to examine factors associated with postdischarge deaths. These included sex, age-group, weight z-score, coexisting conditions (Williams-Beuren syndrome, coronary artery involvement, coarctation, and pulmonary artery stenosis), surgical techniques, and era, defined as early (1982-1995) or late (1996-2003). Survival was assessed by matching with the National Death Index through 2021.
RESULTS: Of 333 patients who met inclusion criteria, 313 (94.0%) survived to discharge and 188 (60.1%) had identifiers for National Death Index matching. Over a median follow-up of 25.2 years (interquartile range, 21.1-29.4 years), 17 deaths occurred. The 30-year survival after discharge from SVAS repair was 88.7% (95% CI, 82.9%-94.8%). Infantile surgery and non-Williams-Beuren syndrome were associated with decreased 30-year survival. From the various repairs, the 2-sinus technique had better outcomes compared with all other types, except the 3-sinus technique (nonsignificant difference). Adjusted analysis revealed infantile age and type of repair as associated with postdischarge probability of death.
CONCLUSIONS: These data demonstrate favorable long-term outcomes after SVAS repair, except for the infantile group that was associated with more diffuse arteriopathy. As techniques continue to evolve, future studies are warranted to investigate their long-term outcomes.