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.