Dee P M, Hubbell M M, Rheuban K S, Tompkins D G, Carpenter M A
Cardiovasc Intervent Radiol. 1981;4(3):158-66. doi: 10.1007/BF02552417.
Congenital absence of the pulmonary valve (CAPV) is a rare anomaly usually associated with ventricular septal defect or Fallot's tetralogy. CAPV may be rapidly fatal in infancy because of severe disturbance of pulmonary ventilation resulting from bronchial compression by massively dilated pulmonary arteries. Other cases may be relatively benign. Our group of five cases with CAPV ranged from the severe to one case in which there was documented closure of a patent ductus arteriosus and a ventricular septal defect, leaving the CAPV as an apparently isolated, well-tolerated lesion. Plain chest radiographs play a major part in the diagnosis of CAPV since they almost invariably give some indication od the aneurysmal dilatation of the pulmonary arteries. On angiography, dilated main pulmonary arteries are shown giving rise to branches of normal or even diminished caliber.
先天性肺动脉瓣缺如（CAPV）是一种罕见的先天性畸形，通常与室间隔缺损或法洛四联症相关。由于巨大扩张的肺动脉压迫支气管导致肺通气严重障碍，CAPV在婴儿期可能迅速致命。其他病例可能相对良性。我们的一组5例CAPV患者病情严重程度不一，其中1例记录显示动脉导管未闭和室间隔缺损闭合，仅留下CAPV作为一个明显孤立且耐受性良好的病变。胸部X线平片在CAPV的诊断中起主要作用，因为它们几乎总是能显示出肺动脉瘤样扩张的一些迹象。在血管造影中，扩张的主肺动脉显示出正常或甚至管径减小的分支。
