完全性房室间隔缺损合并法洛四联症或四联症型右室双出口的解剖矫治

Total repair of atrioventricular septal defect with tetralogy of Fallot or Fallot′s type double outlet right ventricle

目的 总结完全性房室间隔缺损合并法洛四联症或四联症型右室双出口的解剖矫治经验。方法 从 2 0 0 0年 6月至 2 0 0 2年 9月共对 10例完全性房室间隔缺损合并法洛四联症或四联症型右室双出口的患者进行了解剖矫治 ,采用“双片法”修补房室间隔缺损 ,经右房和右室切口修补室缺并重建左室流出道 ,疏通右室流出道 ,根据肺动脉瓣环及肺动脉的发育程度 ,用自体心包作流出道成形 ,冠状动脉异常分布于右室流出道的患者 ,应用带瓣的同种血管行右室到肺动脉的外管道连接。结果 无手术死亡 ,晚期死亡 1例 ,死亡率 10 % ,术后室缺残余漏 2例 ,2次手术修补后 1例痊愈 ,另 1例合并右侧膈肌麻痹 ,3次手术行膈肌折叠后痊愈。结论 完全性房室间隔缺损合并法洛四联症或四联症型右室双出口行一期解剖矫治可获得良好的手术效果 ,远期结果有待进一步随防。

Objective To summarize the experience in total repair of atrioventricular septal defect with tetralogy of Fallot or Fallot′s type double outlet right ventricle. Methods Ten patients with atrioventricular septal defect associated with tetralogy of Fallot or Fallot′s type double outlet right ventricle underwent repair between June 2000 and September 2002. Atrioventricular septal defect was repaired with a 2-patch technique. The ventricular patch material was dacron artificial vascular patch, autologous pericardium was used to close the ostium of atrial septal defect after repair of the atrioventricular valve. Closure of ventricular septal defect (VSD) necessitated a combined right atrial and right ventricular approach. Each patient underwent an extensive infundibulectomy performed through the right ventriculotomy. If the pulmonary valve was more than 1 mm or 2 mm in diameter, smaller than the normal size, a transannular pericardial patch was used. If abnormal coronary arteries existed in the surface of right ventricular outflow, a homograft was used to connect the right ventricle with pulmonary artery. Results There was one death (10%) six months after repair. Two patients were not able to be extubated from ventilator because of residual VSD. After repairing the residual VSD, one patient was extubated from ventilator. The other still wasn′t able to be extubated from ventilator due to right diaphragm paralysis. After folding right diaphragm, the patient got stable recovery. Conclusion Atrioventricular septal defect with tetralogy of Fallot or Fallot′s type double outlet right ventricle can be corrected with low mortality. Residual VSD can severely affect recovery. Long-term results need to be followed up.