单纯性单侧肺动脉发育不全3例报道及文献复习  

Three cases report of isolated unilateral pulmonary artery agenesis and literature review

作　　者：倪崇俊[1] 金建强[1] 冯薇[1] 黄建安[1] 

机构地区：[1]苏州大学附属第一医院呼吸与危重症科,江苏苏州215006

出　　处：《中国现代医药杂志》2017年第11期17-20,共4页Modern Medicine Journal of China

摘　　要：目的探讨成人单纯性单侧肺动脉发育不全的临床及影像学特征。方法回顾性分析临床诊断的3例成人单纯性单侧肺动脉发育不全的临床表现与影像资料,并复习文献。结果 3例患者中2例为左肺动脉缺如,1例为右肺动脉狭窄。临床表现为反复的肺部感染伴进行性活动后胸闷气促,胸部X线常诊断为支气管扩张或肺炎,但一侧肺门影增大及患侧肺血管纹理稀疏有提示作用。超声心动图提示存在肺动脉高压,通过胸部普通CT及增强CT可诊断本病及合并的肺发育不全。结论成人单纯性单侧肺动脉发育不全临床缺乏特异性,漏诊误诊较多。提高对本病胸部X线表现的认识,通过胸部CT及血管造影确诊。Objective To investigate the clinical and imaging characteristics of isolated unilateral pulmonary artery agenesis in adults. Methods The clinical data of 3 cases of isolated unilateral pulmonary artery agenesis were retrospectively analyzed and the related literatures were reviewed. Results 2 cases were absence of left pulmonary artery, the other one case was right pulmonary artery stenosis. Clinical manifestations were repeated lung infection with chest tightness after progressive activity, chest X-ray often diagnosed as bronchiectasis or pneumonia, but the side of the hilar shadow increased and the veins in the side lung were sparse. Echocardiography prompted the presence of pulmonary hypertension, through the ordinary chest CT and enhanced CT could diagnose the disease and the lung dysplasia. Conclusion The clinical manifestation of isolated unilateral artery agenesis in adults is not specific and the clinical misdiagnosis are more. Improve understanding of the chest X-ray performance,and through chest CT and angiography can get the diagnosis.

关 键 词：肺动脉 发育不全 肺动脉缺如 肺动脉狭窄 

分 类 号：R543.2[医药卫生—心血管疾病]