全身性エリテマトーデスに合併した大動脈弁輪拡張症に対するBentall手術の1例

Bentall Procedure for Annuloaortic Ectasia in a Patient with Systemic Lupus Erythematosus；Report of a Case

はじめに　全身性エリテマトーデス（SLE）は，若年女性に多く発症する，自己免疫機序による全身諸臓器の炎症性疾患である1）．しかし，SLE例における心血管系合併症の報告は少ない．われわれは，大動脈弁輪拡張症，大動脈弁閉鎖不全症に対してBentall手術を施行したSLE例を経験したので報告する．

Systemic lupus erythematosus （SLE） is a chronic inflammatory disease, rarely complicated with cardiac valvular abnormality or aortic aneurysm. An asymptomatic 40-year-old woman with a 15-year history of SLE developed annuloaortic ectasia and aortic valve regurgitation. She had long been maintained on steroid therapy for the treatment of SLE. Her serum anti-phospholipid antibodies were positive. An enhanced computed tomography revealed a dilated aortic root and ascending aorta （57 mm in diameter）. Cardiac ultrasonography demonstrated severe aortic regurgitation.

We performed the Bentall procedure using a composite graft with the Carrel patch technique. Histopathological examination of the aortic wall showed cystic medial necrosis without any evidence of vasculitis. Careful long-term follow-up is mandatory for assessing the risk of later anastomotic dehiscence and pseudoaneurysm formation.