Four surgically treated cases of aortic valve regurgitation associated with the aortitis syndrome were reported. All patients were female and ranged in age from 38 to 51 years. In two cases, obstructive lesions of the aortic arch branch were seen in aortograms. Three patients had no inflammatory findings at the operation and one was operated on after improvement of inflammatory findings by steroid therapy. Aortic regurgitation was caused by annuloaortic ectasia, and aortic valve replacement was performed by the everting mattress suture method in all cases. One patient with stenosis of the left carotid artery, occlusion of the left subclavian artery, and hypertension died of extended cerebral damage due to suspected low flow cerebral perfusion during cardiopulmonary bypass. The other three patients are doing well 4 months to 11 years after surgery without paravalvular leakage. In cardiac surgery for the aortitis syndrome with an obstructive lesion of the aortic arch branch, deep hypothermic and high flow cardiopulmonary perfusion is required to prevent ischemic brain complications during surgery.