主动脉瓣上狭窄的外科治疗

Surgical Treatment of Supravalvular Aortic Stenosis

１９０年４月至１９６年１１月收治７例主动脉瓣上狭窄（ＳＶＡＳ）病人，其中４例为Ｗｉｌｉａｍｓ综合征。术前超声心动图测定跨狭窄段压力阶差为８．０～１９．２ｋＰａ（１ｋＰａ＝７．５ｍｍＨｇ），平均１１．０９±４．３ｋＰａ。所有病人均行主动脉扩大补片成形术。结果：无围术期死亡，随访２～８０个月，平均２５．７个月，解除狭窄满意，仅１例出现轻度主动脉瓣关闭不全。结论：运用菱形补片直接进行主动脉扩大成形术仍然是矫治沙漏型ＳＶＡＳ的首选方法。Ｗｉｌｉａｍｓ综合征病人应注意是否合并其他畸形。

Aim:Supravalvular aortic stenosis(SVAS)is a relatively rare type of left ventricular outflow obstruction.This paper reviewed the experience of surgical treatment of this congenital anomaly.Clinical material and method:Form April 1990 to November 1996,seven patients with SVAS underwent extended patch aortoplasty under cardiopulmonary bypass.There were 5 boys and 2 girls aged from 5 to 16 years,with a mean of 10,4 of 7 patients had Williams' syndrome.Postoperative follow up was 2 to 80(mean,25.7)months.Results:There was no postoperative mortality,postoperative morbidity.The mean peak to peak systolic pressure gradient across aortic valve decreased from preoperative reading of 83.4±32.6mmHg to 17.2±9.97mmHg postoperatively.Therefore,satisfactory relief of SVAS was obtained.However mild aortic valve regurgitation was found in one patient during follow up.Conclusion:Extended patch aortoplasty is the choice of treatment of SVAS.Associated heart deformity should be considered in patients with Williams'syndrome.Exchocardiography plays an important role in diagnosing SVAS.