完全閉塞となった異所性大動脈縮窄症の1例

A Case of an Unusual Type of Coarctation of the Aorta Terminating in the Complete Obstruction

　症例は19歳，女性．生後5カ月で心雑音を指摘された．5歳時心臓カテーテル検査を施行され，大動脈縮窄症（Edwards分類IV型C）と診断された．外来で経過観察中に高血圧が出現し，精査加療目的で当科入院となった．入院時現症では身長168cm，体重59 kg，下肢の発育は良好で，上肢の高血圧（180／100，左右差なし）を認めた．胸部では大動脈弁領域でLevine 3°／VIの収縮期雑音を聴取し，大腿動脈の拍動は左右とも触知不能．心臓超音波検査，心臓カテーテル検査より，大動脈二尖弁と下行大動脈での大動脈の中断および側副血行路を介して横隔膜下に低形成の腹部大動脈を認めた．5歳時の診断と病型および病変部位に変化はなかったが，大動脈縮窄部は完全閉塞へと進行していた．経過観察中に大動脈縮窄部位が完全閉塞を来した特異な症例を経験したので，若干の考察を加えて報告した．

A 19 year-old girl with an unusual type of coarctation of the aorta terminating in complete obstruction is presented. At the age of 5 years, she was diagnosed by aortography to have coarctation of the aorta of type Ⅳ C according to Edward's classification. No surgical treatment was performed at that time because her growth was good and she didn't have hypertension. During the follow-up period, marked hypertension developed, and she was readmitted. The aortogram revealed a bicuspid aortic valve and total obstruction of the descending thoracic aorta at the site where coarcta-tion was noticed previously.