Alkaptonuria-associated aortic stenosis

Zoe Lok, Jacob Goldstein, Julian Anderson Smith

Research output: Contribution to journalArticleOther

12 Citations (Scopus)


Alkaptonuria is an autosomal recessive disorder of tyrosine metabolism, which results in accumulation of unmetabolized homogentisic acid and its oxidized product in various tissues, including the heart. Cardiovascular involvement is a rare but serious complication of the disease. We present two patients who have undergone successful aortic valve replacement for alkaptonuria-associated aortic stenosis along with a review of the literature. doi: 10.1111/jocs.12129 (J Card Surg 2013;28:417-420) ? 2013 Wiley Periodicals, Inc.
Original languageEnglish
Pages (from-to)417 - 420
Number of pages4
JournalJournal of Cardiac Surgery
Issue number4
Publication statusPublished - 2013

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