先天性主动脉瓣上狭窄26例临床分析

Clinical analysis of congenital supravalvular aortic stenosis in 26 cases

目的探讨主动脉瓣上狭窄的诊断与治疗。方法收集首都医科大学附属北京安贞医院2002年至2012年主动脉瓣上狭窄26例患儿资料,进行回顾性分析。结果 26例患儿中10例合并智力障碍,12例合并生长发育障碍,2例有家族史,4例合并其他心脏畸形。26例经超声心动图检查证实为主动脉瓣上局限性狭窄。2例患儿行心导管及造影检查,其中1例患儿造影完成后因呼吸、循环衰竭抢救无效死亡。12例患儿行心脏CTA检查。13例患儿行外科手术治疗,11例术后恢复良好,1例死亡,1例术后出现脑部并发症随访。结论超声心动图是诊断主动脉瓣上狭窄的基本方法,必要时结合心脏CTA和(或)心导管造影检查明确诊断。外科手术可矫治主动脉瓣上狭窄,部分合并肺动脉狭窄者可考虑同时矫治,近期效果满意。

Objective To investigate the diagnosis and management of congenital supravalvular aortic stenosis. Meth- ods Data of 26 cases is collected and analyzed retrospectively, among which there were mental retardation in l0 cas- es, growth retardation in 12 cases, positive family history in 2 cases and other heart abnormalities in 4 cases. Results Local supravalvular aortic stenosis was proved in 26 cases by thoracic echocardiography. Catheterization and angiogra- pby was performed in 2 cases, and one patient died because of respiration and circulation failure after angiography. Com- puted tomography angiography was done in 12 cases. Surgical operation was conducted in 13 cases, and l 1 patients re- covered uneventfully. Of the other two patients, one died during operation, and the other was complicated with brain prob- lem. Conclusions Echocardiography is the basic method for diagnosis; computed tomography angiography and/or cathe- terization is needed necessarily in establishing diagnosis. Surgical operation is feasible in correcting supravalvnlar aortic stenosis with satisfied recovery in short-term follow-up, and sometimes pulmonary artery plasty performed if necessary.