Beta-thalassemia cardiomyopathy: history, present considerations, and future perspectives.
Beta-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.
Kremastinos
Dimitrios T
DT
Second Department of Cardiology, Athens University Medical School, Attikon University Hospital, 1 Rimini St., Athens, Greece.
Farmakis
Dimitrios
D
Aessopos
Athanasios
A
Hahalis
George
G
Hamodraka
Eftychia
E
Tsiapras
Dimitrios
D
Keren
Andre
A
D009202:Cardiomyopathies / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D006333:Heart Failure / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D017086:beta-Thalassemia / Q000150:complications* / Q000503:physiopathology / Q000628:therapy