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Rupture of chordae tendineae in patients with -thalassemia

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Farmakis D, Deftereos S, Giakoumis A, Polymeropoulos E, Aessopos A. Rupture of chordae tendineae in patients with -thalassemia.

Eur J Haematol 2004: 72: 296–298. © Blackwell Munksgaard 2004. Abstract:

Cardiac disease is the primary cause of mortality in β-thalassemia patients. Except for ventricular dysfunction and pulmonary hypertension that represent the main forms of heart disease in these patients, valvular abnormalities including valvular regurgitation, endocardial thickening and calcification and mitral valve prolapse have also been described. Here we present two patients with thalassemia major and mitral chordal rupture, a previously undescribed abnormality in this population. Pathogenesis of this finding may involve thalassemia-related pseudoxanthoma elasticum-like syndrome, a diffuse elastic tissue defect, which is observed with a notable frequency in these patients and has been associated with numerous cardiovascular complications, including valvular ones.
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Keywords: pseudoxanthoma elasticum; rupture of cordae tendineae; thalassemia

Document Type: Research Article

Publication date: April 1, 2004

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