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 syn-drome underwent one-stage radical operation under general anesthesia during hypothermic car-diopulmonary 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.Dur-ing operation,gentle operation technique was carried out and appropriate surgical incision and re-pair material were selected to avoid conduction block and other complications.After operation,pa-tients were admitted to CICU,and respiratory tract management and rational use of breathing ma-chine were strengthened.Moreover,vasoactive drugs and antibiotics were rationally used to main-tain 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.Con-clusion 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 chil-dren with ventricular septal defect.
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