摘要目的 总结完全性心内膜垫缺损合并右室双出口双心室修复的经验.方法 2006年7月至209年11月对6例完全性心内膜垫缺损合并右室双出口病人采用双片法修补完全性心内膜垫缺损,人工血管补片作右心室内隧道,连接室间隔缺损与主动脉口,引导左心室血经内隧道进入主动脉;应用牛心包补片扩大右室流出道;肺动脉瓣环小者用带瓣补片跨环修补右室流出道;心室内隧道有可能阻塞右室流出道造成重度梗阻者,右室流出道与主肺动脉之间安置带瓣管道(Rastelli手术).结果 术后死亡1例,占16.6%,其余病例恢复良好.结论 完全性心内膜垫缺损合并右室双出口一期双心室修复可获得良好效果.

abstractsObjective Double-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.Methods From July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.Results One death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.