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英文誌(2004-)

Journal of Medical Ultrasonics

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2013 - Vol.40

Vol.40 No.04

Case Report(症例報告)

(0407 - 0412)

重複僧帽弁口を合併した心室中隔欠損の臨床経過

Clinical course of ventricular septal defect with double-orifice mitral valve

高橋 信1, 佐藤 陽子1, 鳥谷 由貴子2, 中野 智2, 早田 航1, 小山 耕太郎1, 千田 勝一2

Shin TAKAHASHI1, Yoko SATO1, Yukiko TOYA2, Satoshi NAKANO2, Wataru SODA1, Kotaro OYAMA1, Shoichi CHIDA2

1岩手医科大学附属病院循環器小児科, 2岩手医科大学附属病院小児科

1Department of Pediatric Cardiology, Iwate Medical University Hospital, 2Department of Pediatrics, Iwate Medical University Hospital

キーワード : double-orifice mitral valve, ventricular septal defect, relative mitral valve stenosis, echocardiography, cardiac catheterization

今回,心室中隔欠損と僧帽弁狭窄を合併し高度の肺高血圧を来たした11ヵ月の男児を経験した.心臓超音波検査で,大きな膜様部心室中隔欠損とcomplete bridge typeの重複僧帽弁口がみられ,左室流入血流圧較差は増大し,顕性化した僧帽弁狭窄の所見を認めた.心臓カテーテル検査でも高度の肺高血圧を呈し,NO負荷試験で肺血管閉塞性病変は否定した.相対的僧帽弁狭窄の程度が不明のため外科的治療として心室中隔欠損閉鎖術を行った.術後に肺高血圧が軽減し,僧帽弁狭窄の程度は軽度のため経過観察とした.心室内左右短絡を伴った重複僧帽弁口の評価において,術前に様々な方法で有効僧帽弁口面積を推測することが,治療方針決定に重要であり,その方法として心臓超音波検査は有用であった.

An 11-month-old boy who had been diagnosed as having a ventricular septal defect and mitral valve stenosis presented with severe pulmonary hypertension. Echocardiography showed a large perimembranous ventricular septal defect and a complete bridge-type double-orifice mitral valve together with an increased left ventricular inflow pressure gradient and overt mitral valve stenosis. Cardiac catheterization revealed severe pulmonary hypertension, but the nitric oxide tolerance test ruled out pulmonary vascular obstructive disease. The ventricular septal defect was surgically closed because the relative severity of the mitral valve stenosis was unknown. The patient remained under postoperative observation due to improved pulmonary hypertension and mild mitral valve stenosis. During evaluation of the double-orifice mitral valve with intracardiac left-right shunt, it was important in terms of the treatment strategy decision to infer the effective mitral orifice area, and cardiac ultrasonography was useful as the preoperative method.