肺动脉闭锁合并室间隔缺损104例诊断分析

Pulmonary atresia with ventricular septal defect:an analysis of 104 cases

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 。

Objective To analyse the anatomy of pulmonary atresia with ventricular septal defect(PA/VSD).Methods Between June 1992 and May 2002,104 patients with PA/VSD underwent echocardiographic and angiographic examination.Results The atresia occurred at infundibular or pulmonary valvar in 31 cases,at trunk with confluent in 50 cases,the others at left or right or both pulmonary artery with non confluent.The pulmonary blood supplies were the major aorto pulmonary collateral arteries in 51 cases,persistent ductus arteriosus in 27 cases and small systemic collateral arteries in 26 cases.Conclusion The pulmonary atresia may occur in various positions.The pulmonary blood supplies are various.