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Aortic Valve Stenosis with Black Pigmentation Due to Alkaptonuria: A Case Report
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  • Takahiro Shojima,
  • Yasuyuki Zaima,
  • Kazuyoshi Takagi,
  • Tohru Takaseya,
  • Hiroyuki Otsuka,
  • Yusuke Shintani,
  • Takanori Kono,
  • Satoshi Kikusaki,
  • Kosuke Saku,
  • Eiki Tayama
Takahiro Shojima
Kurume University School of Medicine
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Yasuyuki Zaima
Kurume University Hospital
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Kazuyoshi Takagi
Kurume university, school of medicine
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Tohru Takaseya
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Hiroyuki Otsuka
Munakata Suikokai General Hospital
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Yusuke Shintani
Munakata Suikokai General Hospital
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Takanori Kono
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Satoshi Kikusaki
Kurume University School of Medicine
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Kosuke Saku
Kurume University Hospital
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Eiki Tayama
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Abstract

We report about a 78-year-old woman with severe aortic valve stenosis (AS). She had heavily calcified and thickened aortic valve leaflets with black pigmentation. After excision of the aortic valve leaflets, biological aortic valve replacement (AVR) was performed. Alkaptonuria was diagnosed and confirmed based on increased urinary excretion of homogentisic acid. She has had no cardiovascular system-related symptoms for 3 years and no evidence of structural valve deterioration. Data on the long-term outcomes of AVR associated with structural valve deterioration that could regulate prosthetic valve selection in alkaptonuria are limited. Therefore, further research on the natural evolution of AS and the rate of structural valve deterioration after AVR is needed to provide an optimal prosthesis for these patients.