出血性もやもや病を合併した解離性上行大動脈瘤に対する自己弁温存大動脈基部置換術の1例

Valve-sparing Aortic Root Replacement in Patient with Dissecting Ascending Aortic Aneurysm Complicated with Hemorrhagic Moyamoya Disease；Report of a Case

はじめに　もやもや病（MMD）は，内頸動脈の進行性閉塞により形成される側副血行路の血管造影所見から命名された脳血管疾患であり，脳梗塞や脳出血の原因となる難病である1～3）．われわれは，二度の脳出血既往と局所的な脳虚血を有するMMDを合併した，解離性上行大動脈瘤の症例を経験したので報告する．

A 62-year-old female with Moyamoya disease （MMD） had cardiomegaly pointed out by chest X-ray and was admitted to our hospital. Chest computed tomography （CT） scan and echocardiography revealed a large dissecting ascending aortic aneurysm 78 mm in diameter combined with severe aortic regurgitation and mild mitral regurgitation. She had a history of intracranial hemorrhage related with MMD twice. Considering her relatively young age and risk of intracranial hemorrhage, valve-sparing aortic root replacement was planned to avoid anticoagulant therapy. Operation was performed keeping intra-operative blood pressure, perfusion pressure, Paco2 and activating clotting time in appropriate ranges. The postoperative course was uneventful without cerebral complication. No clinical symptom related with MMD was observed in 4 years after the operation.