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Guideline recommendations for heart complications in thalassemia major

Cogliandro, Tiziana; Derchi, Giorgio; Mancuso, Luigi; Mayer, M Carolina; Pannone, Bruno; Pepe, Alessia; Pili, Marcello; Bina, Patrizio; Cianciulli, Paolo; De Sanctis, Vincenzo; Maggio, Aureliounder the auspices of the Society for the Study of Thalassemia and Hemoglobinopathies (SoSTE)

Journal of Cardiovascular Medicine: May 2008 - Volume 9 - Issue 5 - p 515–525
doi: 10.2459/JCM.0b013e3282f20847
Points of view

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.

aUOSC Cardiologia, UOS, Centro della Cardiopatia nelle Talassemie, Osp. S. Eugenio, Roma, Italy

bSC di Cardiologia, EO Ospedali Galliera, Genova, Italy

cUOSC di Cardiologia, Laboratorio di Ecocardiografia, AO V. Cervello, Palermo, Italy

dUOSC 4a Medicina Interna, AORN A. Cardarelli, Napoli, Italy

eMRI Laboratory, Institute of Clinical Physiology, CNR, Pisa, Italy

fServizio di Cardiologia, Ospedale Regionale per le Microcitemie, ASL No. 8, Cagliari, Italy

gUO Talassemia, Osp. S. Eugenio, Roma, Italy

hUO di Pediatria ed Adolescentologia, Azienda Ospedaliera Universitaria, Arcispedale S. Anna, Ferrara, Italy

iUOC di Ematologia II con Talassemia, AO V. Cervello, Palermo, Italy

Received 12 February, 2007

Revised 7 September, 2007

Accepted 11 September, 2007

Correspondence and requests for reprints to Aurelio Maggio, UOC di Ematologia II con Talassemia, AO V. Cervello, Via Trabucco no. 180, 90146 Palermo, Italy Tel: +39 091 688 5251; fax: +39 091 688 0828; e-mail:

Sponsorship: This work was supported by the Foundation ‘Leonardo Giambrone’.

© 2008 Italian Federation of Cardiology. All rights reserved.