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lüll Beta-thalassemia cardiomyopathy: history, present considerations, and future perspectives Kremastinos DT; Farmakis D; Aessopos A; Hahalis G; Hamodraka E; Tsiapras D; Keren ACirc Heart Fail 2010[May]; 3 (3): 451-8Beta-thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia that typically requires life-long transfusion therapy. Although traditionally prevalent in the Mediterranean basin, Middle East, North India, and Southeast Asia, immigration of those populations to North America and Western Europe has rendered beta-thalassemia a global health problem. Cardiac complications represent the primary cause of mortality and one of the major causes of morbidity in those patients. Heart disease is mainly expressed by a particular cardiomyopathy that progressively leads to heart failure and death. The beta-thalassemia cardiomyopathy is mainly characterized by 2 distinct phenotypes, a dilated phenotype, with left ventricular dilatation and impaired contractility and a restrictive phenotype, with restrictive left ventricular filling, pulmonary hypertension, and right heart failure. The pathophysiology of the disorder is multifactorial, with a central role of myocardial iron overload and the significant contribution of immunoinflammatory mechanisms. Patients' management is demanding and requires a multidisciplinary approach, preferably in specialized centers.|Cardiomyopathies/*epidemiology/physiopathology/therapy[MESH]|Heart Failure/*epidemiology/physiopathology/therapy[MESH]|Humans[MESH]|beta-Thalassemia/*complications/physiopathology/therapy[MESH] |