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目的 分析肺动脉闭锁合并室间隔缺损 (PA/VSD)的解剖类型及血流动力学改变 ,探讨其适宜的手术方式。方法 对 1992年 6月至 2 0 0 2年 5月在广东省心血管病研究所儿科住院的PA/VSD患儿共 10 4例 ,采用超声心动图结合心血管造影术 ,确定闭锁的部位、肺动脉的发育情况及血供来源。结果 右室流出道及瓣膜闭锁、有肺动脉总干 31例 ;肺动脉总干闭锁、左右肺动脉有汇合 5 0例 ;左肺动脉闭锁 10例 ,右肺动脉闭锁 8例 ;左右肺动脉均闭锁 5例。肺动脉的血供来源 :大的主肺动脉侧支血管 5 1例 ,动脉导管未闭 2 7例 ,多支小的侧支血管 2 6例。合并畸形有卵圆孔未闭、房间隔缺损、大动脉转位、完全性房室间隔缺损、右室双出口、镜面右位心、右旋心及左旋心。结论 合并室间隔缺损的肺动脉闭锁可发生在不同部位 ,肺动脉的血供来源多样化
Objective To analyze the anatomic types and hemodynamic changes of pulmonary arterial atresia combined with ventricular septal defect (PA / VSD) and to explore its suitable surgical approach. Methods A total of 104 children with PA / VSD hospitalized in Guangdong Provincial Institute of Cardiovascular Disease from June 1992 to May 2002 were enrolled in this study. Echocardiography and cardiovascular angiography were used to determine the location of the atresia. Pulmonary artery development and blood supply. Results Right ventricular outflow tract and valve atresia, there are 31 cases of total pulmonary artery; pulmonary artery total block, the convergence of the left and right pulmonary artery 50 cases; left pulmonary artery occlusion in 10 cases, right pulmonary artery occlusion in 8 cases; left and right pulmonary artery occlusion in 5 cases. Pulmonary artery blood supply: large main pulmonary artery collateral vessels in 51 cases, 27 cases of patent ductus arteriosus, multiple small branches of the blood vessels in 26 cases. Combined deformity foramen ovale, atrial septal defect, aortic transposition, complete atrioventricular septal defect, right ventricular double outlet, mirror right centrum, right ventricle and left ventricle. Conclusions Pulmonary atresia combined with ventricular septal defect can occur in different parts of the pulmonary artery blood supply diversification