成人主動脈嚴重狹窄－－罕見特質病例報告

Severe Coarctation of the Aorta in an Adult- A Case Report of a Rare Entity

先天性主動脈狹窄在嬰兒時期最常見，而且都發生在特定的部位；然而發生在腹部主動脈或是胸腔主動脈則少見，胸腔主動脈缺損更是非常罕見的病例。這篇報告是一位罹患有先天性胸腔主動脈缺陷的女性患者，年齡五十歲，罹患腦中風，動脈血管攝影顯示完全缺少胸部主動脈，而下肢循環完全依靠兩側內乳動脈，同時患者並正常生產數位小孩。而其上下肢有170釐米汞柱之壓力差。以直徑10釐米的人工血管做旁道手術，由左鎖骨下動脈連結到左側股動脈，手術過程相當成功；六個月後，追蹤病患，其血壓、脈搏皆正常，而且上下肢之動脈壓力差爲20釐米汞柱。由於此個案非常少見，其癒後追蹤更是不易得到，所以我們特別報告此罕見病例。

Congenital coarctation of the aorta is frequently seen in infancy, and it is usually located at specific sites. It is unusual for the lesion to be found in the abdominal segment of the aorta or more rarely in the upper thoracic aorta. The absence of the upper thoracic aorta is more rarely seen than are other lesions. We describe a case of congenital absence of the upper thoracic aorta. Our patient was a 50-year-old woman who had a cerebral vascular accident (CVA) and who presented with signs of severe coarctation of the aorta and aortic stenosis. Physical examination showed a pressure gradient of 170 mmHg between the upper and lower extremities. The diagnosis was confirmed by aortography which showed that the blood flow was perfused to the lower extremity through a bilateral internal mammary artery. A good surgical result was achieved using a 10-mm diameter vascutek bypass from the left subclavian artery to the left femoral artery. Six months after the operation, the patient had normal blood pressure and good femoral pulses. The pressure gradient was 20 mmHg between the upper and lower extremities. However, due to the rarity of the disease, information concerning late follow up is not yet available.