Farmakis Dimitrios, Polonifi Aikaterini, Deftereos Spyros, Tsironi Maria, Papaioannou Ioannis, Aessopos Athanasios
First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece.
Ann Thorac Surg. 2006 Feb;81(2):737-9. doi: 10.1016/j.athoracsur.2004.12.003.
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.
