An 80-year-old woman presented with dilatation of the distal aortic arch due to chronic type B aortic dissection. She underwent thoracic endovascular aortic repair (TEVAR) in zone 2 with GORE TAG thoracic endoprostheses (40 mm-15 cm and 34 mm-20 cm) for closure of the entry site at the proximal descending aorta. TEVAR was successfully performed and blood flow in the false lumen stopped. Two months after TEVAR, she was admitted to our hospital owing to syncope. A CT scan revealed type A aortic dissection, and emergency surgery was performed. The entry was proximal to the stent graft, and we performed total arch replacement with preservation of the stent graft. Retrograde type A aortic dissection is a rare but lethal complication of TEVAR. Careful consideration of the device selection is needed, and attention should be paid to the placement of the stent graft.

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.

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.