Abstract
The thalassemia syndromes are associated with cardiovascular complications, which differ with the varying phenotypes encountered. The well-recognized paradigm of heart failure induced by myocardial iron overload, in thalassemia major (TM), has now been joined by pulmonary arterial hypertension (mostly seen in thalassemia intermedia) among other more subtle disorders of the cardiovascular system, including endothelial dysfunction. Cardiovascular magnetic resonance (T2*) has allowed cardiac iron load to be quantified noninvasively, and the routine measurement of T2* has been associated with a marked improvement in the outlook for patients with TM, in particular. Echocardiography remains an indispensable tool in the cardiovascular assessment of patients, it provides many insights into cardiovascular function, and its use allows improved management of patients. It is particularly suited to assess diastolic function, diagnose intracardiac masses (usually thrombus), and assess right ventricular function and pulmonary pressure.
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