title: Aortic valve replacement in a patient with thalassemia intermedia.
abstract: Pseudoxanthoma elasticum-like diffuse elastic tissue defects and hypercoagulability are two well-established features in beta-thalassemia, which play a key role in the development of a spectrum of collateral complications in these patients. We present here a middle-aged thalassemia intermedia patient with severe aortic stenosis caused by heavy calcification of the valve, a hallmark of pseudoxanthoma elasticum. The patient underwent a successful replacement of the valve, which is the first such operation ever reported in these patients. However, rapid thrombosis leaded to failure of the mechanical valve, despite proper antithrombotic therapy.
authors.affiliation: First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece. dfarm1@panafonet.gr
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Farmakis
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Polonifi
authors.forename: Spyros
authors.initials: S
authors.lastname: Deftereos
authors.forename: Maria
authors.initials: M
authors.lastname: Tsironi
authors.forename: Ioannis
authors.initials: I
authors.lastname: Papaioannou
authors.forename: Athanasios
authors.initials: A
authors.lastname: Aessopos
mesh_terms: D001024:Aortic Valve Stenosis / Q000209:etiology* / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008875:Middle Aged; D011475:Prosthesis Failure; D011561:Pseudoxanthoma Elasticum / Q000150:complications / Q000209:etiology; D013927:Thrombosis / Q000150:complications / Q000209:etiology; D017086:beta-Thalassemia / Q000150:complications*