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Rupture of chordae tendineae in patients with β‐thalassemia
Author(s) -
Farmakis Dimitrios,
Deftereos Spyros,
Giakoumis Anastasios,
Polymeropoulos Evangelos,
Aessopos Athanasios
Publication year - 2004
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2004.00213.x
Subject(s) - medicine , thalassemia , cardiology , chordae tendineae , pulmonary hypertension , calcification , mitral regurgitation , disease , beta thalassemia , abnormality , mitral valve prolapse , mitral valve , psychiatry
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.