Rupture of chordae tendineae in patients with β-thalassemia

Autor: Dimitrios Farmakis, Athanasios Aessopos, Spyros Deftereos, Evangelos Polymeropoulos, Anastasios Giakoumis
Rok vydání: 2004
Předmět:
Zdroj: European Journal of Haematology. 72:296-298
ISSN: 0902-4441
DOI: 10.1111/j.1600-0609.2004.00213.x
Popis: 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.
Databáze: OpenAIRE