成人法洛四联症外科治疗的远期疗效  

Long-term Results of Tetralogy of Fallot in Adults

作　　者：纪广玉[1] 徐志云[1] 王志农[1] 邹良建[1] 唐昊[1] 张浩[1] 张宇峰[1] 

机构地区：[1]第二军医大学附属长海医院胸心外科解放军胸心外科研究所,上海200433

出　　处：《中国胸心血管外科临床杂志》2010年第4期287-291,共5页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

摘　　要：目的分析成人法洛四联症(TOF)患者外科治疗的远期疗效,探讨围手术期处理要点。方法自2000年1月至2008年3月第二军医大学附属长海医院共收治年龄≥14岁TOF患者149例,其中男78例,女71例;年龄14～53岁,平均年龄26.3岁。有20例患者先行主肺动脉分流术,再行根治术;129例行一期根治术。右心室流出道拓宽38例,跨瓣补片107例,同种主动脉带瓣管道重建右心室流出道4例。结果全组手术死亡6例,病死率为4.0%(6/149),4例为术后发生严重低心排血量综合征(LCOS)和多器官功能衰竭死亡,2例为严重肾功能衰竭死亡。术后发生胸腔积液11例,灌注肺10例,严重低心排血量综合征9例,严重心律失常7例,再次开胸止血术7例,再次气管内插管6例,室间隔缺损残余漏5例(再次手术行室间隔缺损修补2例、经皮介入封堵2例),肾功能衰竭2例。术后随访134例,随访率93.7%(134/143);随访时间3～102个月(47.2±28.6个月)。晚期死亡2例,1例于术后12个月因继发感染性心内膜炎死亡,1例术后29个月猝死。发生室间隔缺损残余漏1例(2mm小缺损),患者活动和生活正常;肺动脉瓣跨瓣压差大于40mmHg4例,重度肺动脉瓣反流2例;未发现三尖瓣中重度反流。随访期间生存132例,生活质量明显提高,心功能分级(NYHA)Ⅰ级121例,Ⅱ级11例。结论由于长期右心室流出道狭窄和缺氧,成人TOF患者病情较为复杂,术前应全面评估,术后积极治疗并发症,对左心室和肺动脉发育不良的患者应先行分流术;成人TOF根治术后近期和远期效果良好。Objective To evaluate the long-term results of surgical treatment of tetralogy of Fallot （TOF） in adults and discuss the peri-operative treatment skills.Methods From January 2000 to March 2008,149 patients older than 14 years with tetralogy of Fallot received surgical treatment in Changhai Hospital.Among the patients,there were 78 males and 71 females with ages ranged from 14 years to 53 years and the average age was 26.3 years.Twenty patients had previous pulmonary arterial shunts before radical treatment.A total of 129 patients underwent primary radical treatment.Thirty-eight patients received a right ventricular outflow tract patch,107 patients had transannular patch,and 4 patients had homograft aorta with valves.Results Hospital mortality was 4.0%（6/149）.Four patients died of low cardiac output syndrome （LCOS）,and multiple organ failure,and 2 patients died of acute renal failure.The postoperative complications included pleural effusion in 11 patients,pulmonary edema in 10 patients,severe LCOS in 9 patients,severe cardiac arrhythmia in 7 patients,reoperation for excessive bleeding in 7 patients,reintubation in 6 patients,and residual ventricular septal defect （VSD） in 5 patients （two of them had reoperation for residual VSD repair and 2 received transcatheter closure of VSD）.One hundred and thirty-four patients were followed up for 3 to 102 months （47.2±28.6 months） with a follow-up rate of 93.7%（134/143）.Late death occurred in 2 patients,one of whom died of secondary infective endocarditis and the other had a sudden death 29 months after operation.During the follow-up,one patient had residual VSD （2 mm）,but had a normal life.The peak systolic right ventricle-to-pulmonary artery pressure gradient exceeded 40 mmHg in 4 patients.Two patients had severe pulmonary regurgitation.A total of 132 patients survived and had an improved life.One hundred and twenty-one patients had class Ⅰ heart function （NYHA）,and 11 patients in class Ⅱ.Conclusion The pathophysiologic conditions o

关 键 词：法洛四联症 成人 外科治疗 远期疗效 

分 类 号：R654.2[医药卫生—外科学]