Division of Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital, Little Rock, Arkansas 72202, USA.
Ann Thorac Surg. 2010 Dec;90(6):2016-22. doi: 10.1016/j.athoracsur.2010.07.086.
Supravalvular aortic stenosis (SVAS) is the rarest type of left ventricular outflow tract obstruction. We reviewed our experience with this anomaly and analyzed risk factors for death or reoperation.
Between 1984 and 2009, 49 patients had surgery for SVAS. A single-patch technique was used in 3, two-sinus enlargement in 39, and three-sinus enlargement in 7. Variables evaluated included age at surgery (<2 versus >2 years old), presence of pulmonary artery stenosis, type of SVAS (focal versus diffuse), presence of valvular aortic stenosis, and era of surgery.
The only early death occurred in a patient who experienced cardiac arrest during anesthesia induction and could not be separated from bypass after surgery. There were 2 late deaths at 3 and 11 years after SVAS repair, both related to treatment for pulmonary artery stenosis. Actuarial survival at 5, 10, and 20 years was 95%, 95%, and 90%, respectively. Sixteen patients required 23 reoperations: for pulmonary artery stenosis (n = 10), distal aortic stenosis (n = 9), aortic valve stenosis (n = 4), and coronary artery stenosis (n = 1). Actuarial reoperation-free survivals at 5, 10, and 20 years were 73%, 58%, and 52%, respectively. Coexistent pulmonary artery stenosis, young age at surgery, and diffuse type SVAS were predictors of lower freedom from death or reoperation by both univariate and multivariate analyses.
Survival after surgical repair of SVAS is excellent. However, reoperation is frequent, especially when the patients also have pulmonary artery stenosis, diffuse type SVAS, and initial surgery at a young age.
主动脉瓣上狭窄(SVAS)是左心室流出道梗阻中最罕见的类型。我们回顾了我们在这种异常情况下的经验,并分析了导致死亡或再次手术的危险因素。
1984 年至 2009 年间,49 例患者因 SVAS 接受了手术。3 例采用单补丁技术,39 例采用双窦扩大,7 例采用三窦扩大。评估的变量包括手术时的年龄(<2 岁与>2 岁)、肺动脉狭窄的存在、SVAS 的类型(局灶性与弥漫性)、主动脉瓣狭窄的存在以及手术的时代。
唯一的早期死亡发生在一名患者身上,该患者在麻醉诱导期间发生心脏骤停,手术后无法与体外循环分离。SVAS 修复后 3 年和 11 年分别有 2 例晚期死亡,均与肺动脉狭窄的治疗有关。5、10 和 20 年的存活率分别为 95%、95%和 90%。16 例患者需要 23 次再次手术:肺动脉狭窄(n=10)、远端主动脉狭窄(n=9)、主动脉瓣狭窄(n=4)和冠状动脉狭窄(n=1)。5、10 和 20 年的无再手术存活率分别为 73%、58%和 52%。多因素分析表明,肺动脉狭窄、手术时年龄较小和弥漫性 SVAS 并存是死亡或再次手术率较低的预测因素。
SVAS 手术后的生存率很高。然而,再次手术的频率很高,尤其是当患者还伴有肺动脉狭窄、弥漫性 SVAS 和早期手术时。