法洛四联症合并肺动脉闭锁外科治疗早期疗效分析

目的:探讨法洛四联症合并肺动脉闭锁(TOF-PA)外科治疗早期疗效。方法:回顾性分析1984年6月至2009年12月,我科对58例TOF合并PA外科治疗的临床资料,其中男性27例,女性31例;年龄6个月～29岁,平均(9.14±5.25)岁。按Castaneda分型,Ⅰ型28例、Ⅱ型11例、Ⅲ型9例及Ⅳ型10例。本组49例采用全麻中度低温体外循环下进行一期矫治手术,Ⅰ型采用跨瓣环的带单瓣的人工血管补片加宽,Ⅱ型采用右心室到肺动脉带瓣管道,Ⅲ型和Ⅳ型采用胸骨正中切口一期单源化和心内修复术。11例在全麻下行主动脉-肺动脉分流术。结果:术后早期并发症:心律失常9例、早期低氧血症7例、严重低心排出量综合征(低心排)5例、血红蛋白尿3例、灌注肺3例、气胸3例、多器官功能衰竭1例、毛细血管渗漏综合征1例、感染1例、肺不张1例及二次开胸止血1例。术后早期死亡10例,病死率5.8%,其余患者均治愈出院。术后1个月～15.5年复查,发绀消失,体质量明显增加。彩超复查未见残余分流,右心室流出道未见梗阻,肺动脉与右心室流出道之间无明显压力阶差。X线检查两肺血均有明显的增加,分布均匀对称。结论:TOF-PA一经诊断应尽快手术,加强术后监护及综合治疗措施,及时纠正术后低心排,积极防治术后心律失常等并发症,均为提高手术成功率的诸因素。外科手术应早期手术病死率低,中、远期心功能效果良好。

The early efficacy of surgical treatment of tetralogy of Fallot with pulmonary atresia

Objective:To probe the early efficacy of Surgical treatment of tetralogy of Fallot with pulmonary atresia.Methods:The 58 patients with tetralogy of Fallot with pulmonary atresia including 27 male and 31 female with age from 6 months to 29 years(mean 9.14±5.25) were studied from June 1984 to December 2009.28 patients were in type Ⅰ TOF-PA,type Ⅱ 11,type Ⅲ 9 and type Ⅳ 10.Through a standard median sternotomy incision.49 patients were operated on under moderate hypothermia and cardiopulmonary bypass.An artificial vessel patch with a monocuspid valve was applied to enlarge at he outlet of right ventricle in type Ⅰ patients and a conduit with valves from right ventricle to pulmonary artery was applied in type Ⅱ patients.Midline one-stage complete unifocalization and repair of ventricular septal defect and major aortopulmonary collaterals were used in patients with type Ⅲ and Ⅳ.11 patients were operated on under moderate hypothermia and cardiopulmonary bypass.Central aorto-pulmonary shunts were performed in 11 cases.Results:The early postoperative complications included arrhythmia in 9 cases,7 cases of early hypoxemia,severe low cardiac output syndrome in 5 cases,3 cases of hemoglobinuria,perfusion lung in 3 cases,pneumothorax 3 cases,multiple organ failure in 1 case,the capillary leak syndrome in 1 case,infection in 1 case,atelectasis in 1 case,the second thoracotomy to stop bleeding in 1.10 patients died early after operation,mortality 5.8%,and The survivors were cured.The survivors were followed up from 1 month to 15.5 year,cyanosis disappeared,body weight increased significantly.Ultrasound review showed that all patiens were without residual shunt,right ventricular outflow tract obstruction,pulmonary artery and right ventricular outflow tract without significant pressure gradient.X-ray examination showed that blood lungs increased significantly,symmetrical distribution.Conclusion:Surgical treatment of patients with TOF-PA can be achieved with lower operative mortality and good medial-term outcome.Strict,intensive surveillance and promptly synthesized treatment for low cardiac output syndrome,cardiac arrhythmia can improve the operative results.Early therapy may effectively assure the successes of the operations.