儿童主动脉瓣上狭窄的外科治疗

Surgical repair of congenital supravalvular aortic stenosis in children

目的总结先天性主动脉瓣上狭窄(SVAS)外科治疗体会。方法选择2003年3月至2010年5月收治的7例SVAS患儿,均在体外循环下以自体心包片用单片法加宽主动脉。结果 7例患儿体外循环时间61～98min,平均(75.9±12.2)min;主动脉阻断时间35～63min,平均(46.2±9.7)min。本组手术无严重并发症及死亡,术后超声心动图检查左室——主动脉压差10～21mmHg,平均(13.3±3.8)mmHg,无主动脉瓣关闭不全。结论主动脉单片法加宽治疗局限性SVAS可以获得理想的治疗效果。对William综合征患儿术前应进行更为详细的检查,了解是否合并其他心血管畸形。

Objective To review the experience of surgical repair of congenital supravalvular aortic stenosis （SVAS）. Methods Between March 2003 and May 2010, aortic orifice enlargement was performed by single-patch technique with auto -pericardial patch under cardiopulmonary bypass in 7 cases with SVAS. Results The duration of cardiopulmonary bypass procedure were 61～98 min, with the mean of （75.9±12.2）min. The aortic cross-clamp time was 35～ 63 min with the mean of 46.2±9.7 min. There was no postoperative mortality and postoperative morbidity. Residual gradients were 10～21mmHg, with the mean of （13.3±3.8） mmHg. No aortic regurgitation occurred. Conclusion The ideal results could be obtained by single -patch enlargement of aorta to discrete SVAS. Associated cardiovascular abnormality should be considered in patients with Williams’syndrome.