Roman J, Jones S
Division of Pulmonary and Critical Care Medicine, Atlanta Veterans Affairs Medical Center, Emory University School of Medicine, Georgia 30033.
Am J Med Sci. 1995 Mar;309(3):188-90. doi: 10.1097/00000441-199503000-00011.
Unilateral absence of the left pulmonary artery is a rare congenital anomaly that may remain unrecognized until adulthood, in which case it may lead to inappropriate diagnosis and management. The authors describe a case in which a patient came to their institution with dyspnea and a chest roentgenogram showing a prominent right pulmonary artery, right aortic arch, and a left lung mass accompanied by ipsilateral volume loss. Although the initial evaluation suggested a differential diagnosis that included thromboembolic pulmonary disease and vascular compression by tumor, further workup revealed unilateral absence of the left pulmonary artery accompanied by left lung hypoplasia, and interestingly, ipsilateral emphysema and adenocarcinoma. The embryologic factors responsible for this abnormality and how it may potentially affect the development of emphysema and adenocarcinoma are discussed.
左肺动脉单侧缺如是一种罕见的先天性异常，可能直到成年才被发现，在这种情况下可能导致不恰当的诊断和治疗。作者描述了一例患者，该患者因呼吸困难前来就诊，胸部X线片显示右肺动脉突出、右主动脉弓以及左肺肿块伴同侧肺容积减小。尽管初始评估提示鉴别诊断包括血栓栓塞性肺疾病和肿瘤导致的血管受压，但进一步检查发现左肺动脉单侧缺如伴左肺发育不全，有趣的是，还有同侧肺气肿和腺癌。本文讨论了导致这种异常的胚胎学因素以及它可能如何潜在地影响肺气肿和腺癌的发生发展。
