異型大動脈縮窄症に心室中隔欠損症と胸部大動脈瘤を合併した1症例

A case of atypical coarctation of aorta with a chest aneurysm and ventricular septal defect

　今回我々は小児期に心室中隔欠損症単独奇形と診断された先天性異型大動脈縮窄症が胸部大動脈瘤までを合併した18歳の女性例を経験し，きわめてまれであると思われたので，文献的考察や現在までの報告例を調べて紹介する。術前診断は大動脈造影が有効であったが，非観血的な方法として，超音波エコー法も有用であった。術式はDacron人工血管を用いて大動脈間を置換，また左鎖骨下動脈-大動脈間もこれを用いて置換した。

An 18 year-old woman under observation for ventricular septal defect was also diagnosed for a rare congenital atypical coarctation of the aorta and aneurysm in the region of chest. The contrast of the aorta as well as ultrasonic echocardiography was effective for an early diagnosis before surgery.

Among the variations of atypical coarctation of the aorta, malformation is rarely observed. This is the 9 th case in which it was located between the left common carotid artery and the left subcla-vian artery. Complications of the ventricular septal defect and atypical coarctation of the aorta was noted, but in theses cases protorusive deviation and posterior deviation were not recognized. Though there are many causes for atypical coarctation of the aorta, this case seemed to be in a poststenotic dilatation state. A Dacron artificial blood vessel was used as a bypath on the aorta and also to connect the aorta and the left subclavian artery. The postoperation recovery was good and the pres-sure gradient was reduced. Although this case was reported to be the 14th in which ventriculer septal defect was diagnosed with congenital atypical coarc-tation of the aorta, it is the 6th case when such a similar variation as mentioned was found. It is considered to be the first case in Japan which developed further into complications of ventricular septal defect.