小儿法乐四联症围术期的治疗策略

Perioperative Management of Tetralogy of Fallot in Children

目的总结法乐四联症（TOF）患儿手术治疗经验，探讨手术时机、手术适应证和围术期处理。方法回顾性分析我科2004年1月至2010年6月收治的132例小儿TOF的临床资料，其中男98例，女34例；年龄4个月至12岁，平均12．85岁。术前均行超声心动图或心导管造影检查确诊，均在体外循环下行根治性手术，部分病例以自体心包或带瓣自体心包扩大右室流出道和（或）肺动脉，对合并畸形作相应处理。结果本组围术期死亡4例（3．03％），其中2例死于术后多器官功能衰竭，2例死于低心排出量综合征。随访129例，随访时间12～90个月；失访3例。6例残余微量分流，生长发育正常，无不适。结论对症状显著的TOF患儿应尽早手术矫治，提高手术水平，加强术后监护是确保手术成功的关键。

Objective To summary the experience of primary correction of Fallot （TOF） in children, and describe the optimal times and the indication, as well as perioperative management. Methods Clinical materi- al of 132 consecutive patients with TOF who were undergoing early surgery from January 2004 to June 2010 were retrospectively reviewed. There were 98 males and 34 females with mean age of 12.85 months（4months- 12years）. All patients were diagnosed by echocardiogram or cardioangiography and all the corrective operation were performed under cardiopulmonary bypass （ CPB ）, autologous pericardial patch, or with valve, was used to enlarge the right ventricular outflow tract and/or the pulmonary trunk, and the accompanied malformations Were repaired at the same time. Results 4 patients died, the cause of death were postoperative infection with multi- ple organ failul （2 cases） , low cardiac output syndrome （2 cases）. 129 patients were followed up from 12 months to 90 mionths. 3 patients lost to follow - up. 6 trivial residual shunt, All eases were satisfactory. Conclusion- s hffants with symptomatic TOF should be repaired early. Improve the operative technique and emphasize the postoperative management take an important role in increase the outcome of surgical treatment.