Congenital Absence of the Right Pulmonary Artery: Four Cases Report

先天性右肺主動脈發育不全：四病例報告

惲純和(Chun-Ho Yun)；許清寅(Chin-Yin Sheu)；施焄鏻(Shin-Lin Shih)

血管攝影 ； 電腦斷層掃描 ； 先天性右肺主動脈發育不全 ； Angiography ； Computed tomography ； Right pulmonary artery anomaly

中華放射線醫學雜誌

29卷1期（2004 / 02 / 01）

35 - 40

英文

先天性右肺主動脈發育不全是一種罕見的先天性疾病。我們在此報告四例患有先天性右肺主動脈發育不全的病例，並回顧相關文獻。在影像學方面最主要的表徵為右側肺容積減少。其他的表徵則包含總氣管和縱隔腔移位，右側橫隔膜升高，右側正常的肺血管陰影減少。電腦斷層掃描和血管攝影能使我們精確地診斷此疾病。然而，胸部X光攝影和電腦斷層掃描便足以提供既安全且非侵入性的方式來做正確的診斷。

Congenital absence of the right pulmonary artery is an uncommon congenital disorder. We describe the clinical and radiologic presentations of this disease in 4 cases (3 men, 1woman; mean age: 22.8y/o) in our hospital. Decrease of the right lung volume is the most common radiologic finding. Other characteristic findings include trachea and mediastinum shift, right hemidiaphragm elevation and loss of normal right pulmonary vascularity. CT scan and angiography enable us to confirm the diagnosis precisely. However, plain film and CT scan offer a safer and non-invasive way to make the diagnosis.