单侧肺静脉闭锁2例并文献复习

Unilateral Pulmonary Vein Atresia: Two Cases Reports and Literature Review

单侧肺静脉闭锁是一种罕见病,多为先天性,缺乏特异性临床表现,常见临床表现包括反复肺部感染、咯血、呼吸困难及活动耐量减低等,其中以咯血较为常见。目前,临床尚缺乏单侧肺静脉闭锁的标准治疗方案,而单侧肺静脉闭锁患者若未得到及时有效治疗,则病死率达50%以上。本文报道了2例单侧肺静脉闭锁患儿,并结合相关文献进行了复习,旨在提高临床对该病的认识及诊治水平。

Unilateral pulmonary vein atresia is one kind of rare diseases,mainly were congenital; clinical manifestations of unilateral pulmonary vein atresia are not specific, common clinical manifestations include recurrent lung infection,hemoptysis,dyspnea and reduce of activity tolerance,thereinto hemoptysis is the most common symptom. There was no standard regimen for unilateral pulmonary vein atresia so far,and the fatality rate was over 50% in unilateral pulmonary vein atresia patients without prompt and effective treatment. This paper reported two children with unilateral pulmonary vein atresia,and reviewed pertinent literatures,in order to improve the understanding,diagnosis and treatment level on clinic.