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Ann Thorac Surg 2006;81:737-739
© 2006 The Society of Thoracic Surgeons

Case report

Aortic Valve Replacement in a Patient With Thalassemia Intermedia

Dimitrios Farmakis, MD, PhD a , * , Aikaterini Polonifi, BS a , Spyros Deftereos, MD, PhD a , Maria Tsironi, MD, PhD a , Ioannis Papaioannou, MD b , Athanasios Aessopos a

a First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece
b Department of Cardiac Surgery, Ippokratio Hospital, Athens, Greece

Accepted for publication December 10, 2004.

* Address correspondence to Dr Farmakis, 182 Kallergi St, Piraeus, 185 44 Greece (Email: dfarm1{at}panafonet.gr).

Pseudoxanthoma elasticum-like diffuse elastic tissue defects and hypercoagulability are two well-established features in ß-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.




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A. Aessopos, M. Kati, and D. Farmakis
Heart disease in thalassemia intermedia: a review of the underlying pathophysiology
Haematologica, May 1, 2007; 92(5): 658 - 665.
[Abstract] [Full Text] [PDF]


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