title: Black aorta in a patient with alkaptonuria (ochronosis).
abstract: A rare cause of valvular heart disease is the deposition of foreign material in the valvular tissues, including material accumulating as a result of inborn errors of metabolism of the essential amino acids. Alkaptonuria can result in accumulation of homogentisic acid. We report the case of a patient with alkaptonuria undergoing surgery for aortic valve replacement.
authors.affiliation: Department of Cardiac Surgery and Heart Transplantation, San Camillo Hospital, Circonvallazione Gianicolense 1, Rome, Italy. gioconci@libero.it
authors.forename: Giovanni
authors.initials: G
authors.lastname: Concistrè
authors.forename: Brenno
authors.initials: B
authors.lastname: Fiorani
authors.forename: Federico
authors.initials: F
authors.lastname: Ranocchi
authors.forename: Giovanni
authors.initials: G
authors.lastname: Casali
authors.forename: Antonio
authors.initials: A
authors.lastname: Loforte
authors.forename: Francesco
authors.initials: F
authors.lastname: Musumeci
mesh_terms: D000474:Alkaptonuria / Q000150:complications* / Q000378:metabolism*; D001024:Aortic Valve Stenosis / Q000209:etiology* / Q000378:metabolism / Q000601:surgery; D005260:Female; D019918:Heart Valve Prosthesis Implantation; D006713:Homogentisic Acid / Q000378:metabolism; D006801:Humans; D008875:Middle Aged; D009794:Ochronosis / Q000209:etiology* / Q000378:metabolism / Q000601:surgery
chemical_list: D006713:Homogentisic Acid