先天性主动脉瓣上狭窄的外科治疗

Surgical repair of congenital supravalvular aortic stenosis

目的总结6例先天性主动脉辫上狭窄(SVAS)术前诊断和外科治疗中的经验、教训和疗效。方法2003年1月至2008年6月收治SVAS患者6例,男性3例,女性3例,年龄1.9～19.0(5.73±6.36)岁,局限性狭窄5例,弥漫性狭窄1例。Williams综合征3例,3例并发其他心脏畸形,其中1例同时合并动脉导管未闭、主动脉瓣下狭窄、主动脉瓣两叶畸形、二尖瓣狭窄关闭不全和细菌性心内膜炎,1例合并动脉导管未闭,还有1例合并肺动脉狭窄。术前超声心动图检查均有初步诊断,有2例患者手术前做了左心导管和造影,3例行64排螺旋CT扫描,对诊断和手术方案的确定很有意义。手术前检查发现跨狭窄环的压差为45～121mmHg。手术方法:单片法加宽3例,倒"Y"法3例,同期行主动脉瓣置换、二尖瓣置换、左室流出道疏通和动脉导管结扎1例,行肺动脉加宽1例,行动脉导管缝闭1例。结果本组手术无死亡,除合并细菌性心内膜炎及多种畸形的患者术后出现低心排和呼吸功能不全和感染,其余患者恢复顺利,所有患者痊愈出院。手术后有1例虽然主动脉瓣上狭窄解除理想,但是发现主动脉弓有弥漫性轻度狭窄。结论SVAS经术前超声心动图检查基本可以诊断,对Williams综合征患者术前应进行更为详细的检查,如果有条件应该考虑行无创伤的64排螺旋CT检查,可以发现合并的畸形如主动脉弓的病变,肺动脉狭窄等;一旦发现主动脉辫上狭窄应该积极手术治疗,否则可能引起心律失常和猝死。SVAS的手术治疗效果良好,目前不主张应用单片法进行矫治。

Objective To review the experience and curative effect of surgical repair of congenital supravalvnlar aortic stenosis（SVAS）. Methods From January 2003 to June 2008, 6 cases suffering from congenital SVAS underwent surgical correction at Shenzhen Sun Yet-sen Cardiovascular Hospital. There were 3 males and 3 females ranging from 1.9-19,0（5.73±6.36） years old. Of the 5 cases were localized type SVAS and one was diffused type SVAS. And 3 cases combined with Williams syndrome, 3 cases with other cardiac defects, one with PDA, 1 case with pulmonary stenosis, another with PDA, subaortic stenosis bicuspid aortic vulve,mitral valve stenosis and regurgitation and bacteria endocarditis. All pateints were diagnosed by echocardiography study preoperatively. There were two caeses undergoing catheter and radiography and three cases using 64-Slice Spiral Computed Tomographic Angiography. The pressure gradient was from 45 mm Hg to 121 mm Hg preoperatively. Operative procedures included single, diamond-shaped patch aortoplasty in 3 cases, inverted bifurcated patch aortoplasty in 3 cases, of one associated with aortic vulve replacement, the subaortic membrane dissection and PDA ligation, pulmoaery plasty in 1cases. Results There was no death. Only one suffered from low cardic output and respiration disfuction in postoperation. Other pateints recovered smoothly. Conclusion Good surgical outcome of congenital SVAS can be achieved with the patch-aortoplasty technique. The congenital SVAS can be diagnosed by means of eehoeardiography study easily and early. The 64-slice spiral CT angiography and radiography can reveal type of SVAS and be of benefit to operation.