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Case Reports
. 2018 Jul 3;26(3):467-469.
doi: 10.5606/tgkdc.dergisi.2018.15552. eCollection 2018 Jul.

A rare cause of aortic valve stenosis: Ochronosis

Atakan Atalay  1 , Vural Polat  1 , Nesrin Turhan  2 , İrfan Taşoğlu  1
Affiliations
Case Reports

A rare cause of aortic valve stenosis: Ochronosis

Atakan Atalay et al. Turk Gogus Kalp Damar Cerrahisi Derg. .

Abstract
in English, Turkish

Ochronosis is an autosomal recessive metabolic disorder which may cause aortic valve stenosis. In this article, we report a 58-yearold male patient with alkaptonuria who presented to our institute with severe aortic valve stenosis. He underwent successful aortic valve replacement with a mechanical prosthesis. The deposition of foreign material in valvular tissue is a rare case scenario which may result in serious problems, the management of which may necessitate valve replacement with prosthetic heart valves.

Okronozis, aort kapak darlığına neden olabilen bir otozomal resesif metabolik bozukluktur. Bu yazıda, ciddi aort kapak darlığı nedeniyle kliniğimize başvuran alkaptonürisi olan 58 yaşında bir erkek hasta sunuldu. Hastaya mekanik protez ile başarılı aort kapak replasmanı yapıldı. Kapak dokusunda yabancı maddelerin birikimi ciddi sorunlara yol açan nadir durumdur ve yönetimi kalp kapak protezi ile kapak replasmanı gerektirebilir.

Keywords: Alkaptonuria; aortic valve; ochronosis.

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Conflict of interest statement

Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Figures

Figure 1
Figure 1. Photograph of operative specimen shows black discoloration of the aortic valve.
Figure 2
Figure 2. Photomicrograph of a section of valvular leaflet shows ochronotic pigment deposition (H-E×100).
See this image and copyright information in PMC

References

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