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Updated: Nov 27, 2025

Full-root Aortic Valve Replacement by Stentless Aortic Xenografts in Patients with Small Aortic Roots
Published on: May 21, 2017
[Ochronosis of the Entire Aortic Root in Alkaptonuria;Report of a Case]
Hiroaki Komatsu1, Hitoshi Kanamitsu, Makoto Mohri
1Department of Cardiovascular Surgery, Japanese Red Cross Society Himeji Hospital, Himeji, Japan.
Abstract:
Alkaptonuria is a rare hereditary disorder of phenylalanine and tyrosine metabolism, which results in ochronosis of cardiovascular structures including valves, aortic intima, and coronary arteries. Aortic valve disease is the most frequently reported cardiac sequela of alkaptonuria. We report a case of 77-year-old woman with known alkaptonuria who underwent aortic valve replacement for severe aortic stenosis. Operative findings showed impressive ochronosis of the aortic valve and the aortic intima. The post-operative course was uneventful and she was discharged 25 days after the surgery.
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