A 71-year-old woman underwent mitral valve replacement with Carpentier-Edwards Pericardial Xenograft for mitral regurgitation on January 8, 1987. She had nocturnal hematuria and paroxysmal nocturnal hemoglobinuria was diagnosed in November, 1992. She had congestive heart failure in February, 1993. Cineradiographical analysis revealed a fracture of the wireform in three locations of the Xenograft and the stent was distorted inward. A second mitral valve replacement was successfully performed on March 16, 1993. She was discharged 45 days after operation after an uneventful course.

A 31-year-old woman complained of dizziness. Preoperative aortogram revealed a saccular type of aneurysm of the aortic arch and abnormal origin of the left subclavian artery. She underwent reconstruction of the aortic arch through 4th intercostal thoracotomy on August 10th, 1996. The aneurysm originated distally to the left common carotid artery and its wall was very thin. Aortic arch replacement with woven Dacron prosthesis was performed between the left common carotid artery and the left subclavian artery using the inclusion method under hypothermic circulatory arrest. The post operative course was uneventful. Cystic medial necrosis of the aneurysmal wall, and normal arterial findings of the left femoral artery were recognized by the pathohistological examinations. Kinking of the aorta is a congenital disease due to abnormal embryonal development. The aortic arch elongates between the left common carotid artery and the left subclavian artery, and arch aneurysm originates in this portion, because the aneurysmal wall is very thin and fragile when accompanied with cystic medial necrosis. Therefore, it is important that the left subclavian artery originating distally to the arch aneurysm in this category of the aortic arch aneurysm be recognized. There are 11 cases which were successfully operated for aortic arch aneurysm associated with congenital kinking of the aorta in Japan.