Nagare Ketak, Idhrees Mohammed, Ibrahim Mohammed, Jacob Aju, Velayudhan Bashi
Institute of Cardiac and Aortic Disorders, SRM Institutes for Medical Science (SIMS Hospitals), Chennai, 600 026 India.
Indian J Thorac Cardiovasc Surg. 2021 May;37(3):320-322. doi: 10.1007/s12055-020-01059-z. Epub 2020 Oct 2.
The commonest cardiac pathology in patients with alkaptonuria is aortic stenosis. Patients with alkaptonuria and aortic stenosis may remain asymptomatic until the 6th decade. Surgeons may have to deal with per-operative difficulties as alkaptonuria is a systemic disease. Proper preoperative planning is important. The mechanical valve prosthesis is advisable in a patient with alkaptonuria and aortic stenosis considering disease pathophysiology. We report a 70-year-old male diagnosed with alkaptonuria and aortic stenosis, who underwent aortic valve (mechanical valve prosthesis) and ascending aorta replacement.
黑尿症患者最常见的心脏病理改变是主动脉狭窄。黑尿症合并主动脉狭窄的患者可能直到60岁都没有症状。由于黑尿症是一种全身性疾病，外科医生可能不得不应对手术中的困难。进行适当的术前规划很重要。考虑到疾病的病理生理学，对于黑尿症合并主动脉狭窄的患者，建议使用机械瓣膜假体。我们报告了一名70岁男性，诊断为黑尿症合并主动脉狭窄，他接受了主动脉瓣（机械瓣膜假体）置换和升主动脉置换术。
