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Rupture of chordae tendineae in patients with beta-thalassemia.

Abstract：

:Cardiac disease is the primary cause of mortality in beta-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.

journal_name

Eur J Haematol

journal_title

European journal of haematology

authors

Farmakis D,Deftereos S,Giakoumis A,Polymeropoulos E,Aessopos A

doi

10.1111/j.1600-0609.2004.00213.x

subject

Has Abstract

pub_date

2004-04-01 00:00:00

pages

296-8

issue

4

eissn

0902-4441

issn

1600-0609

pii

EJH213

journal_volume

72

pub_type

杂志文章

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