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先天性肺动脉瓣缺如或发育不良仅有遗迹,是少见的先天性心脏病,约占先心的0.3％,经常单独存在,亦可合并室间隔缺损和肺动脉瓣环狭窄,成为法乐四联症的一种特殊类型,还可合并动脉导管未闭、房间隔缺损,大血管易位及主动脉弓异常等畸形,组成各种复杂先心病。我科曾收2例,其中1例肺动脉瓣缺如,合并室缺、房缺和右室双出口,生前确诊,并经尸解证实。现报告如下:
Congenital pulmonary valve defects or dysplasia only traces of congenital heart disease is rare, about preoccupied 0.3%, often alone, can also be combined ventricular septal defect and pulmonary valve stenosis, as the quadruple A special type of disease, but also the merger of patent ductus arteriosus, atrial septal defect, major vessel transposition and abnormal aortic arch and other deformities, composed of a variety of complex congenital heart disease. My department had received 2 cases, of which 1 case of pulmonary valve absent, combined with atrial septal defect, atrial septal defect and right ventricle double exit, diagnosed before his death, confirmed by autopsy. The report is as follows: