右肺動脈缺失合併心室中隔缺損：一病例報告

Absence of Right Pulmonary Artery with Ventricular Septal Defect: Report of a Case

羅韻珊(Yun-Shan Lo) ； 吳俊仁(Jiunn-Ren Wu) ； 黃德揚(Teh-Yang Huang) ； 王仁榮(Jen-Zone Wang)

《Acta Paediatrica Sinica》 29卷5期 (1988/10) Pp. 352-356

摘要

單側性肺動脈缺失是少見的先天性肺血管發育異常。病例為13個月大女嬰，因長期咳嗽及呼吸道感染而住院。理學檢查發現右胸骨凹陷、右側呼吸音減低、右胸骨下緣有第3級收縮期吹氣狀心雜音。胸部X光檢查發現右肺白濁、間有少許肺囊腫、左邊肺氣腫，第六胸脊椎骨半脊椎骨症。經雙相心臟超音波心圖及左心室血管攝影證實有心室中隔缺損。主肺動脈血管攝影發現右肺動脈無顯像。再經胸部電腦斷層攝影確定右肺動脈缺失及右肺發育不全的診斷。病兒在症狀治療後情況改善而出院。

並列摘要

Absence of the pulmonary artery with pulmonary hypoplasia is an uncommon congenital anomaly. It occurs commonly in association with other disorders, such as diaphragmatic hernia, Scimitar syndrome, renal agenesis and dysplasia. There is no specific clinical symptom and it may pass unnoticed until adulthood.
A female baby, 13-month-old, was admitted to our hospital due to persistent cough and frequent upper respiratory tract infection. Physical examination showed mild contraction of right thorax, decreased right breathing sounds and a GrⅢ/VI systolic blowing murmur audible over right sternal border. The chest X-ray film showed ”white out” of right lung field with some pneumatoceles and compensatory emphysematous change of the left lung. A 2-dimensional echocardiography and left ventricular angiography revealed a subaortic ventricular septal defect. The main pulmonary angiography revealed normal visualization of the left pulmonary artery but absence of the right pulmonary artery. The chest computerized tomography scan showed decreasing lung volume of the right side and compensatory emphysematous change with herniation of the left side. The mediastinum and the descending aorta were shifted to the right side. The vascularity of the affected lung was decreased and absence of the right pulmonary artery was found. The patient was under supportive therapy and discharged with good condition.

並列關鍵字

absence of the right pulmonary arter ； pulmonary artery angiography ； computerized tomography ； ventricular s eptal defect