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先天性主动脉瓣狭窄伴左冠窦发育不全。主动脉根部的解剖重建。

Congenital aortic stenosis with hypoplasia of the left sinus of Valsalva. Anatomic reconstruction of the aortic root.

作者信息

Harlan J L, Clark E B, Doty D B

出版信息

J Thorac Cardiovasc Surg. 1985 Feb;89(2):288-94.

PMID:3968912
Abstract

Evaluation of the aortic root in 13 patients with congenital aortic stenosis aged 2.5 to 24 years (mean 8.3 years) has revealed morphologic characteristics of asymmetry of the aortic root caused by a small (hypoplastic) left sinus of Valsalva associated with a supravalvular ridge above the left coronary ostium and dysplasia of the aortic valve. The asymmetry resulted in folding and buckling of the left aortic cusp. The aortic valve was classified as bicuspid in 11 of the 13 patients. Preoperative aortography was characteristic and revealed the diagnosis in all patients. The average left ventricle-aorta systolic pressure gradient was 81 mm Hg. Operative repair consisted of an oblique aortotomy extended in a spiral fashion to the right and posteriorly into the left sinus of Valsalva. Seven patients had further mobilization of the posterior commissure with a second incision to the right of the commissure into the noncoronary sinus. Slightly fused valve commissures were opened in 12 patients. Aortic root reconstruction was accomplished with a spiral Dacron patch and posterior commissural repositioning. Follow-up catheterization at 9 to 35 months (mean 24 months) in five patients demonstrated an average outflow tract systolic gradient of 28 mm Hg and a more symmetrical appearance of the aortic root.

摘要

对13例年龄在2.5至24岁(平均8.3岁)的先天性主动脉瓣狭窄患者的主动脉根部进行评估,发现主动脉根部形态特征不对称,这是由于小(发育不全)的左冠窦,伴有左冠状动脉口上方的瓣上嵴和主动脉瓣发育异常所致。这种不对称导致左主动脉瓣叶折叠和弯曲。13例患者中有11例主动脉瓣被分类为二叶式。术前主动脉造影具有特征性,所有患者均得以确诊。左心室-主动脉收缩压平均梯度为81毫米汞柱。手术修复包括斜行主动脉切开术,以螺旋方式向右和向后延伸至左冠窦。7例患者通过在瓣叶联合右侧向无冠窦做第二个切口进一步游离后瓣叶联合。12例患者打开了轻度融合的瓣膜联合。用螺旋状涤纶补片和后瓣叶联合重新定位完成主动脉根部重建。5例患者在9至35个月(平均24个月)时进行的随访心导管检查显示,平均流出道收缩压梯度为28毫米汞柱,主动脉根部外观更对称。

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