孤立性片側肺動脈欠損症健側肺に発生した転移性肺腫瘍の切除経験

Surgery for a Metastatic Tumor in the Right Lung in Case of Congenital Left Pulmonary Artery Defect

片側肺動脈欠損症は患側近位部肺動脈が欠損するまれな先天性奇形である．心奇形を合併する症例では肺高血圧症も合併して予後不良であるが，心奇形を合併しない孤立性片側肺動脈欠損症は無症状に経過し，偶発的に検診などで発見される例も報告されている．われわれは，孤立性片側肺動脈欠損症の健側肺に発生した転移性肺腫瘍に対し，肺切除術を施行した非常にまれな1例を経験したので報告する．

Unilateral absence of a pulmonary artery （UAPA） is a rare congenital cardiovascular malformation and clinical experience of the surgery for this disease are limiting. We report a case of 62-year-old woman of UAPA （absence of left pulmonary artery） underwent a wedge resection of right lung for metastatic lung cancer. The surgery was performed under double lung ventilation because abnormal lung does not contribute to gas exchange. We were able to perform a wedge resection easily by setting the thoracotomy wound right above the tumor in the spine position. In a performing thoracic surgery under double lung ventilation, the position of surgical wound is very important. Therefore, although the diagnosis of this disease is difficult for the rarity and its nonspecific presentation, preoperative diagnosis of UAPA is very important.