Valve-Sparing Excision of Multiple Papillary Fibroelastomas in the Aortic Valve and Left Ventricular Outflow Tract / 日本心臓血管外科学会雑誌

Primary cardiac fibroelastoma is a relatively rare tumor and is often detected incidentally by echocardiography. We report a case of multiple fibroelastomas that were found incidentally by follow-up echocardiography for hypertrophic cardiomyopathy and were treated with valve-sparing excision. The patient was a 71-year-old man, in whom a 10-mm tumor on the ventricular septum below the right coronary cusp and 3-mm tumors on the left ventricular side of the left and right coronary cusps were detected. Although he had no symptoms, because the tumors were mobile, surgery was performed for preventing embolization and making a definitive diagnosis. The tumor on the ventricular septum was excised together with the surrounding endocardium and part of the myocardium. The tumors on the valve leaflets were excised with the aortic valve cusps spared. Histopathologically, all the tumors were fibroelastomas. The postoperative course was uneventful.

A Case of Partial Remodeling for Type A Aortic Dissection Requiring Aortic Root Reconstruction / 日本心臓血管外科学会雑誌

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 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.