Rupture of chordae tendineae in patients with beta-thalassemia.
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.
Farmakis
Dimitrios
D
First Department of Internal Medicine, University of Athens Medical School, "Laiko" General Hospital, Athens, Greece.
Deftereos
Spyros
S
Giakoumis
Anastasios
A
Polymeropoulos
Evangelos
E
Aessopos
Athanasios
A
D000328:Adult; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000209:etiology; D002815:Chordae Tendineae / Q000000981:diagnostic imaging / Q000473:pathology*; D004452:Echocardiography; D004547:Elastic Tissue / Q000473:pathology; D005260:Female; D006331:Heart Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D008297:Male; D008944:Mitral Valve Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology*; D012422:Rupture, Spontaneous; D017086:beta-Thalassemia / Q000150:complications*