A Severe Aortic Stenosis and Coronary Calcifications in Alkaptonuria / 日本心臓血管外科学会雑誌

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.

Determination of Clamp Site with Reference to a Cerebral Blood Flow Monitoring System in a Patient with a Right Subclavian Artery Aneurysm Complicated by Right Internal Carotid Artery Occlusion / 日本心臓血管外科学会雑誌

A 66-year-old man was referred to our hospital for the treatment of a right subclavian artery aneurysm. Computed tomography showed a 35-mm aneurysm in the right subclavian artery, and occlusion of the right internal carotid artery. A surgical approach with maintenance of intracranial blood flow was required. During anastomosis of the graft to the native vessel, we clamped the distal and proximal portions of the right subclavian artery, to maintain the blood flow of the right common carotid artery. The INVOS® cerebral oximeter (Somanetics Corp., Troy, MI, USA) was useful in determining the clamping site and surgical strategy. The procedure was completed without complications, and the patient was discharged from the hospital on postoperative day 8 following a good clinical course.

Stentgraft Treatment for Inflammatory Aortic Aneurysm of Thoracic Aorta in Behçet's Disease / 日本心臓血管外科学会雑誌

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.

Successful Treatment of Prosthetic Graft Infection 7 Years after Ascending Aorta Replacement and Aortic Root Replacement with a Freestyle Stentless Valve / 日本心臓血管外科学会雑誌

A 62 year-old man presented with severe septic shock complicated by prosthetic graft infection, 7 years after aortic root replacement with a Freestyle stentless valve and graft replacement of the ascending aorta. We initially managed the patient with antimicrobial therapy for 2 months and subsequently surgery was performed, replacing the infected aortic graft with rifampicin-bonded prostheses, and added omentopexy. The infection was cured and has not recurred.