ABSTRACT: Thalassemia major is an inherited hemoglobin disorder resulting in a chronic hemolytic anemia. Transfusion therapy together with elevated gastrointestinal absorption of iron determines iron overload, which causes most of the mortality and morbidity associated with the disease. Heart complications represent the leading cause of mortality in this disease, although, because of an improvement in chelation treatment, an important and progressive increase of life expectancy mainly as a result of a reduction in mortality due to cardiac dysfunction has been demonstrated in recent years. Clinical pictures of heart damage range from the involvement of the ventricles to pulmonary hypertension or symptomatic ventricular or supra-ventricular arrhythmias. For this reason, the possibility of having specific recommendations is noteworthy. These recommendations outline the definition, the follow-up and the treatment of the main heart complications in this group of patients. The identification of topics and the nomination of the committee were made on behalf of the Society for the Study of Thalassemia and Hemoglobinopathies (SoSTE). The document obtained the auspices of ANMCO, SIC, SIRM and the Cardiovascular Magnetic Resonance Working Groups of the ANMCO, SIC and SIRM. All recommendations provided in this document have been performed according to the American Cardiology College (ACC) and American Heart Association (AHA) guidelines. Moreover, the recommendations were reviewed by two external referees before the definitive approval.
Journal of Cardiovascular Medicine 06/2008; 9(5):515-25. · 1.51 Impact Factor