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The heart in transfusion dependent homozygous thalassaemia today – prediction, prevention and management
Author(s) -
Aessopos Athanassios,
Berdoukas Vasilios,
Tsironi Maria
Publication year - 2008
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2007.01018.x
Subject(s) - chelation therapy , medicine , heart failure , intensive care medicine , disease , deferiprone , disease management , presentation (obstetrics) , medical therapy , cardiac magnetic resonance , cardiology , magnetic resonance imaging , thalassemia , surgery , radiology , parkinson's disease
Cardiac disease remains the major cause of death in thalassaemia major. This review deals with the mechanisms involved in heart failure development, the peculiar clinical presentation of congestive heart failure and provides guidelines for diagnosis and management of the acute phase of cardiac failure. It emphasizes the need for intensive medical – cardiac care and aggressive iron chelating management as, with such approaches, today, the patients outcomes can be favourable in the long term. It covers advances in the assessment of cardiac iron overload with the use of magnetic resonance imaging and makes recommendations for preventing the onset of cardiac problems by tailoring iron chelation therapy appropriate to the degree of cardiac iron loading found.