成人和青少年法洛四联症的外科治疗

Surgical treatment of tetralogy of Fallot in adults and adolescents

目的 总结成人和青少年法洛四联症（tetralogy of Fallot,TOF）的外科治疗经验,探讨围手术期处理要点和观察外科疗效.方法 回顾性分析自2000年1月至2014年11月,上海交通大学医学院附属新华医院收治的141例年龄≥12岁法洛四联症患者,其中男性92例、女性49例,年龄12～59岁,平均年龄25.4岁.117例行法洛四联症根治术,右室流出道重建时使用跨肺动脉瓣补片100例,单纯右室流出道扩宽17例.有24例患者一期行体肺分流术（合并肺动脉闭锁17例）,其中15例患者在术后1～3年行二期根治手术,余9例因肺动脉发育不佳未行根治术.观察并随访患者术后近期及中远期外科治疗效果.结果 术后早期（30 d内）死亡4例,病死率2.8％.死亡患者中3例死于低心排出量综合征,1例死于心律失常.术后灌注肺合并肺部感染5例,再次气管插管3例,急性肾功能衰竭需行血透者1例,再次开胸止血2例,胸腔积液21例.随访136例,随访时间3个月至14年,平均（43.7±9.6）个月.三尖瓣重度反流6例,4例顺利再次手术行三尖瓣修复,2例继续观察中;三尖瓣轻至中度反流9例,中度肺动脉残余梗阻3例,小的室间隔残余分流4例,无中远期死亡.结论 成人和青少年TOF患者需行外科手术治疗,通过合理的术前评估,妥善的围手术期处理,存活者远期治疗效果较为满意.

Objective To summarize the experice of surgical treatment of tetralogy of Fallot （TOF） in adults and youngsters and discuss the pert-operative treatment skills and evaluate the long-term results. Methods From January 2000 to November 2014, 141 patients older than 12 years with tetralogy of Failot received surgical treatment in Xinhua Hospital. Among the patients, there were 92 males and 49 females with ages ranging from 12 years to 59 years and the average age was 25.4 years. 117 patients underwent primary radical treatment. Among then] 100 patients had transannular patch, 17 patients received a right ventricular outflow tract patch. 24 patients underwent pulmonary arterial shunts, among whom 15 patients with pulmonary atresia received secondary operation. The left 9 patients did not undergo radical treatment due to the poor development of pulmonary artery. Results Hospital mortality was 2.8%（4/141 ）. 3 patients died of low cardiac output syndrome （LCOS）, and 1 patient died of arrhythmia. The postoperative complications included perfusion lung accompanied with pulmonary infection, rein- tubation in 3 patients, acute renal failure with the need of hemodialysis in 1 patient, reoperation for postoperative bleeding in 2 patients and pleural effusion in 21 patients. 136 patients were followed up for 3 to 168 months（43.7± 9.6 ）months. 6 patients had severe tricuspid regurgitation, among whom 4 patients received reoperation for the re- pair of tricuspid valve and the other 2 patients proceeded the follow-up. Mild and moderate tricuspid regurgitation occurred in 9 patients, moderate residual pulmonary obstruction in 3 patients and 4 patients had residual ventricular septal defect. There was no late death. Conclusion Adult and adolescent patients with TOF are bound to receive surgical treatment. The operational morality is relatively higher than infant patients and more postoperative complications occur. However, with proper perioperative management, repair of TOF in adults and adolescents has acceptable long-term outcomes.