儿童先天性主动脉瓣上狭窄的外科治疗

Surgical treatment for children with supravalvular aortic stenosis

目的回顾总结儿童先天性主动脉瓣上狭窄（SVAS）的外科治疗经验，提高治疗水平。方法1990年至2006年10月25例先天性主动脉瓣上狭窄患儿接受手术治疗。其中男16例，女9例，手术平均年龄（5．4±2．4）岁，平均体重（12．6±3．5）kg。影像学检查发现22例为局限性狭窄，主动脉瓣上0．5～2．0cm处有狭窄段，3例为弥漫性狭窄。平均压力阶差（81．7±21．5）mmHg，William＇s综合征12例。所有患儿均在体外循环下行补片扩大主动脉成形术，11例以“泪珠”状补片扩大成形修补，14例以“裤衩”状补片扩大加宽主动脉狭窄部。补片材料选择应用自体心包17例，Dacron涤纶补片2例，Gore-tex人工血管补片6例，其中内衬自体心包片1例。对伴发畸形同时加以纠治。结果术后检查主动脉瓣上流速、压差均明显下降，平均（26．0±13．2）mmHg，住院死亡1例，存活24例。结论应用“泪珠”状和“裤衩”状补片进行主动脉扩大成形术矫治儿童主动脉瓣上狭窄有效，尤以“裤衩”状补片为首选，婴幼儿补片材料以自体心包为宜。

Objective To summarize the experience of surgical treatment for patients with congenital supravalvular artic stenosis （SVAS） retrospectively. Methods From1990 to 2006,25 cases （16 boys and 9 girls） underwent surgical correction for congenital SVAS at Shanghai Children＇s Medical Center, with the mean age of （5.4 ±2. 4）years and mean body weight of （12. 6 ± 3. 5）kg. Twenty-two cases were diagnosed as SVAS with stenosis at 0. 5 to 2. 0 em above the aortic valve, in whom 3 as the diffused type. The mean gradient pressure of SVAS was （81.7 ± 21.5）mmHg. Twelve cases complicated with William＇s syndrome. Aortoplasty with single teardrop-shaped patch was performed on 11 cases, and aortoplasty with inverted bifurcated patch on 14 cases. Both procedures were performed under extracorporeal circulation. The stuff of patch included autografts of pericardium for 17 cases, Darcon patches for 2 and Gore-tex patches for 6. Results The mean postoperative gradient pressure was （25.96 ± 13. 18）mmHg, which was significantly lower than mean preoperative pressure. The mean postoperative supra-valvular flow rate also decreased significantly. Total mortality during hospitalization was 4%, since one patient died due to postoperative heart failure, while the other 24 survived. Conclusions The aortoplasty with single teardrop-shaped or inverted bifurcated patches is an effective procedure for children with congenital supra-valvular artic stenosis. The inverted bifurcated patch is the primary alternative of aortoplasty for children, while the autograft of pericardium is the primary alternative for infants with SVAS.