Down综合征患儿室间隔缺损的手术治疗

Surgical Treatment of Ventricular Septal Defect in Children with Down Syndrome

目的总结Down综合征患儿室间隔缺损的外科治疗经验。方法 48例Down综合征患儿室间隔缺损在全身麻醉低温体外循环下行一期根治术。术前对于合并肺部感染、心功能不全、肺动脉高压者,给予有效的抗感染、强心及利尿剂、降肺动脉压等治疗;加强静脉营养,提高机体免疫力。术中提高手术技巧,操作轻柔,选择合适的手术切口及修补材料,避免发生传导阻滞等并发症。术后入CICU,加强呼吸道管理,合理使用呼吸机;合理使用血管活性药物,维持循环系统的稳定;合理使用抗生素,减少肺部感染的发生。结果 48例中无1例死亡,并发呼吸机相关性肺炎10例,心律失常2例,气胸1例,心包填塞1例,低心排1例。术后随访6个月至6年,生长发育好,无死亡。结论 Down综合征室间隔缺损患儿应尽早手术,选择适当的手术时机,合理有效的围术期处理,可提高手术成功率,延长患儿寿命。

Objective To summarize the experience with the surgical treatment of ventricular septal defect in children with Down syndrome.Methods Forty-eight children with Down syndrome underwent one-stage radical operation under general anesthesia during hypothermic cardiopulmonary bypass.Patients with lung infection,heart failure,and pulmonary hypertension were given anti-infection,cardiotonics and diuretics,and anti-pulmonary hypertension,respectively.In addition,intravenous nutrition was strengthened and immunity was improved in all patients.During operation,gentle operation technique was carried out and appropriate surgical incision and repair material were selected to avoid conduction block and other complications.After operation,patients were admitted to CICU,and respiratory tract management and rational use of breathing machine were strengthened.Moreover,vasoactive drugs and antibiotics were rationally used to maintain the stability of circulatory system and to reduce the incidence of pulmonary infection. Results Among the 48 children,ventilator-associated pneumonia occurred in 10,arrhythmia in 2, pneumothorax in 1,pericardial tamponade in 1,and low cardiac output in 1.These children had normal growth and development and no children died after 6 months to 6 years of follow-up.Conclusion Early operation,appropriate surgery timing and reasonable and effective perioperative treatment can improve the success rate of operation and prolong the life of Down syndrome children with ventricular septal defect.