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

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.