Otto D, von Falkenhausen U, Schellong S, Klein H, Welte T
Klinik für Diagnostische Radiologie, Zentrums für Radiologie, Otto-von-Guericke-Universität Magdeburg.
Pneumologie. 1998 Mar;52(3):135-9.
Unilateral pulmonary vein atresia is a rare condition in adults that may cause serious diagnostic problems because of unspecific clinical findings. A 28-year old female patient is described who presented with signs of recurrent pulmonary embolism with shortness of breathing, unilateral thoracic pain and several episodes of haemoptysis. Ventilation and perfusion scans showed a total lack of perfusion in the right lung with only slight disturbance of ventilation. However, no marked increase of pulmonary artery pressure and no signs of a recent thrombosis of peripheral veins were found. Transoesophageal echocardiography and pulmonary angiography in combination with aortography revealed the diagnosis of unilateral pulmonary vein atresia with abnormal branches of bronchial arteries. There is a considerably left-to-right shunt with return of flow from the bronchial arteries to the right and afterwards to the left pulmonary artery. Clinical, radiological and nuclear medical findings as well as therapeutical options are described.
成人单侧肺静脉闭锁是一种罕见疾病，因其临床症状不具特异性，可能导致严重的诊断难题。本文描述了一名28岁女性患者，她表现出反复肺栓塞的症状，包括呼吸急促、单侧胸痛和多次咯血。通气和灌注扫描显示右肺完全缺乏灌注，仅通气略有紊乱。然而，未发现肺动脉压力明显升高，也未发现外周静脉近期血栓形成的迹象。经食管超声心动图、肺血管造影联合主动脉造影确诊为单侧肺静脉闭锁合并支气管动脉分支异常。存在明显的左向右分流，血流从支气管动脉回流至右肺，然后再至左肺动脉。文中描述了临床、放射学和核医学检查结果以及治疗方案。
