小儿永存动脉干外科诊治19例

Surgical correction of 19 children with persistent truncus arteriosus

目的 总结 19例小儿永存动脉干 (PTA)外科治疗的经验。方法 1989年 1月至 2 0 0 2年 6月外科手术纠治 19例PTA。病儿 2月龄～ 5岁 ;体重 3.5～ 16 0kg。按VanPraagh分型 ,A1型 8例 ,A2型9例 ,A3及A4型各 1例。右室 -肺动脉流出道重建用涤纶生物瓣管道和同种带瓣肺动脉各 2例 ,同种带瓣主动脉 11例 ,近 1年的 4例未用外管道。结果 手术死亡率 10 5 %( 2 19例 )。晚期死亡 1例。结论 PTA易早期并发肺血管梗阻性疾病 ,应在 1岁以内行纠治术。手术需防止室间隔缺损残余分流 ;离断肺动脉和修复动脉干缺损时 ,应避免损伤动脉干、瓣膜及冠状动脉口 ;如条件许可 ,A1型及A2型可不用外管道重建右室 -肺动脉流出道 ,避免了因更换管道而再次手术。

Objective: To review the experience of surgical correction of persistent truncus arteriosus(PTA). Methods: From Jan. 1989 through June 2002, nineteen children with PTA, aged 2 months to 5 years, weighed 3.5 to 16 kg, underwent surgical correction. There were 8 cases with type A1, 9 type A2, 1 type A3 and 1 A4 according to Van Praagh's classification. In reconstruction of right ventricle to pulmonary outflow tract, Dacron valved conduit was used in 2 cases, pulmonary homograft in 2, and aortic homograft in 11. Recent one year, homograft was not used in 4 cases. Results: The operative mortality rate was 10.5%(2/19). Late death occurred in one case. Conclusion: PTA has a higher risk to develop pulmonary vascular obstructive disease early and correction should be done before one year of age. From the surgical point of view, following points should be noted: (1) Residual VSD has to be avoided. (2) Careful attention to the truncal valve and coronary ostia is critical when the pulmonary artery is dissected and the truncal defect is repaired. (3) If in some type A1 and A2 patients, homograft may not be used to reconstruct the right ventricle to pulmonary outflow tract, to prevent from reoperation for revision of the conduit.