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症例は43歳,女性.1990年にベーチェット病と診断され近医に通院中であった.高度房室ブロックに伴う失神が出現し恒久的ペースメーカが植え込まれた約8カ月後に呼吸困難で入院した.胸部X線写真では心拡大と肺うっ血を認め,心エコー上8カ月前はごく軽度であった大動脈弁からの逆流シグナルを高度に認めた.経食道エコーでは心室中隔が瘤状に膨隆し右冠尖のバルサルバ洞動脈瘤を認めた.大動脈弁弁輪,心室中隔にベーチェット病の炎症が及び,房室ブロック,大動脈弁閉鎖不全症が相次いで発症したと思われた.ベーチェット病の患者において伝導障害を有する例では他の心病変の出現を念頭におくことが重要と考えられた.
A-43-year-old woman was admitted to our hospital because of syncope. She had been treated with nonster-oidal anti-inflammatory drugs for Behçet's disease for 5 years. Electrocardiogram showed advanced atrio-ventricular block and a permanent pacemaker was emergently implanted. Eight months later, she was read-mitted because of congestive heart failure. Diastolic blowing murmur was newly audible and moderate aor-tic regurgitation signal was detected with doppler echocardiogram. Transesophageal echocardiogram revealed a deformation in the upper part of the inter-ventricular septum to which the right coronary cusp dropped and non-coaptation of the aortic valve from which fact the regurgitant signal originated. Progres-sion of inflammatory change on the upper part of inter-ventricular septum involving the conduction system and aortic annulus was thought to cause advanced atriog ventricular block and aortic regurgitation.
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