A 48-year-old woman was admitted to our hospital with exertional dyspnea and lower leg edema since 2 months previously. Echocardiogram presented dilation of Valsalva sinus, severe AR (aortic regurgitation) and a supra-annular flap. Enhanced cardiac cycle-gated computed tomography revealed Stanford type A aortic dissection. Primary entry was found just above the aortic valve, the right coronary artery branched from the false lumen, and the commissure between the right and non-coronary cusps was detached. The left coronary artery branched from the true lumen. The false lumen was all patent to the bilateral bifurcations of the common iliac artery. We performed valve sparing partial root remodeling, right coronary artery bypass and total arch replacement after the heart failure management. The operation, cardiopulmonary bypass, aortic cross clamp and selective cerebral perfusion times were 402, 234, 167 and 109 min, respectively. The postoperative course was uneventful, and the patient was discharged 12 days after the operation without any complication. Postoperative CT revealed a well-shaped Valsalva and complete thrombosis of the false lumen on the thoracic aorta. Aortic regurgitation completely disappeared according to a postoperative echocardiogram.

A 71-year-old man with Behçet's disease was admitted to our hospital for treatment of a thoracic aortic aneurysm. On admission, there was marked inflammatory response, but blood culture was negative and there was no significant accumulation of gallium scintigraphy. The aorta was shaggy and there were two aneurysms in the descending aorta. We performed endovascular aortic repair for this aneurysm in consideration of the inflammatory aortic aneurysm. After treatment, the patient had paraparesis, however he underwent physical rehabilitation to regain function. He was followed up for 1 year and remains in good clinical condition without anastomotic aneurysm, dilatation or aneurysm at another site.

Alkaptonuria is a rare genetic disease, in which amino acids and tyrosine cannot be processed. A 72-year-old man with a history of aortic valve stenosis presented with coronary 3-vessel disease. Intraoperative findings included ochronosis, which is pigmentation caused by the accumulation of homogentistic acids in connective tissues, or on the severely calcified aortic valve, the intima of the aorta, and the coronary arteries. The pigmented region of the coronary arteries had significant stenosis. Aortic valve replacement and coronary artery bypass were performed. From these findings and his past history of arthritis, we diagnosed alkaptonuria. The patient had an uneventful recovery.