Unilateral Absence of Right Pulmonary Artery in an Asymptomatic Adolescent: A Case Report and Literature Review

一名無症狀青少年的單側又肺動脈先天性缺失－病例報告與文獻回顧

吳寬澧(Kuan-Li Wu) ； 蔡怡萱(Yi-Hsuan Tsai) ； 蔡明儒(Ming-Ju Tsai) ； 鍾飲文(Inn-Wen Chong)

《胸腔醫學》 30卷1期 (2015/02) Pp. 42-47

摘要

單側肺動脈缺失是一種罕見的先天性異常。部分患者在幼兒時期就產生致命性的併發症，然而也有部分的病人持續無症狀至成人階段。因此通常是在例行的胸腔X光或電腦斷層檢查時意外發現。可能的症狀包含咳血、呼吸困難、反覆性下呼吸道感染等。此外高海拔肺水腫也在此類病人中被報導過。影像學上的特徵為單側發育不全的肺合併有縱膈腔偏移。在此，我們報告一位十五歲的健康男性經由核磁共振血管造影診斷為右側肺動脈缺失且有左下肺靜脈狹窄，確認並無合併先天性心臟病。而合併肺靜脈狹窄目前在文獻上未曾提及。由於病人拒絕進一步侵入性檢查，仍持續在門診追蹤當中。

關鍵字

單側肺動脈缺失 ； 肺發育不全 ； 肺靜脈狹窄

並列摘要

Unilateral absence of pulmonary artery (UAPA) is a rare congenital malformation. Some cases cause deadly complications during childhood, others are usually diagnosed incidentally in asymptomatic patients in adulthood by chest plain film. The image feature on plain film is often a hypoplastic lung on the affected side. Confirmatory CT angiography or magnetic resonance angiography may be needed. The common symptoms of UAPA are hemoptysis, dyspnea, and recurrent pulmonary infections. However, high-altitude pulmonary edema has been thought to be a possible complication recently. Herein, we present an asymptomatic 15-year-old adolescent with absence of a right pulmonary artery. Magnetic resonance angiography confirmed this diagnosis and revealed associated left inferior pulmonary vein stenosis, which has not been reported previously.

並列關鍵字

unilateral absence of pulmonary artery ； pulmonary hypoplasia ； pulmonary vein stenosis