法洛四联症合并肺动脉闭锁的外科治疗

目的探讨法洛四联症合并肺动脉闭锁(TOF-PA)的外科治疗，重点讨论不同类型的手术时机和方法及适应证。方法1984年6月至2003年12月对24例TOF—PA病儿进行矫治手术，其中男10例，女14例；年龄6个月～9岁；Ⅰ型14例，Ⅱ型8例，Ⅲ型和Ⅳ型各1例。全组均采用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TOF-PA采用胸骨正中切口一期单源化和心内修复术。结果术后早期死亡4例，其中Ⅰ型、Ⅱ型各2例，死因为严重低心排血量综合征2例、灌注肺和多脏器功能衰竭各1例。Ⅰ型和Ⅱ型长期随访16例，随访1个月～15．5年，NYHA心功能Ⅰ或Ⅱ级14例，Ⅲ和Ⅳ级各1例；Ⅲ型和Ⅳ型分别随访1个月和6个月，效果满意。结论TOF-PA一经诊断应尽快手术，条件较好的早期行矫治手术可得到满意的效果。

Surgical treatment of tetralogy of Fallot with pulmonary atresia

Objective To evaluate the experiences of complete repair of tetralogy of Fallot with pulmonary atresia (TOF-PA). Methods From June1984 to December 2003, 24 patients with TOF-PA underwent complete surgical repair. There were ten males and fourteen females. The age ranged from 6 months to 9 years. 14 patients were in type I TOF-PA, type II 8, type III and type IV 1 each. Through a standard median sternotomy incision, all patients were operated on under moderate hypothermia and cardiopulmonary bypass. An artificial vessel patch with a monocuspid valve was applied to enlarged the outlet of right ventricle in typeIpatients and a conduit with valves from right ventricle to pulmonary artery was applied in typeII 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. Results Early mortality was 13.3% (4 patients). The causes of death were severe low cardiac output (2 patients), respiratory function failure (1 patient) and multiorgan function failure (1 patient). There was no late death. 18 patients were followed up from one month to 15.5 years. Postoperative heart function (NYHA) was class I or II in 16 patients and class III or IV in 2. Conclusion Surgical repair of patients with TOF-PA can be achieved with acceptable mortality and good results. In selected patients one stage surgical correction can be done through a midline sternotomy approach.