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Review
. 2009 Apr;25(4):213-8.
doi: 10.1016/s0828-282x(09)70064-9.

Iron overload thalassemic cardiomyopathy: iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity

Suree Lekawanvijit  1 , Nipon Chattipakorn
Affiliations
Review

Iron overload thalassemic cardiomyopathy: iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity

Suree Lekawanvijit et al. Can J Cardiol. 2009 Apr.

Abstract
in English, French

Patients with thalassemia major have inevitably suffered from complications of the disease, due to iron overload. Among such complications, cardiomyopathy is the leading cause of morbidity and mortality (63.6% to 71%). The major causes of death in this group of patients are congestive heart failure and fatal cardiac tachyarrhythmias leading to sudden cardiac death. The free radical-mediated pathway is the principal mechanism of iron toxicity. The consequent series of events caused by iron overload lead to catastrophic cardiac effects. The authors review the electrophysiological and molecular mechanisms, pathophysiology and correlated clinical insight of heart failure and arrhythmias in iron overload thalassemic cardiomyopathy.

Les patients atteints de thalassémie majeure ont inévitablement souffert de complications de la maladie en raison d’une surcharge en fer. Parmi ces complications, la myocardiopathie est la principale cause de morbidité et de mortalité (63,6 % à 71 %). Les principales causes de décès au sein de ce groupe de patients sont l’insuffisance cardiaque congestive et les tachyarythmies cardiaques fatales. La voie à médiation par radicaux libres est le principal mécanisme de toxicité ferreuse. La série d’événements causés par la surcharge en fer a des effets cardiaques catastrophiques. Les auteurs ont examiné les mécanismes électrophysiologiques et moléculaires, la physiopathologie et les aperçus cliniques connexes d’insuffisance cardiaque et d’arythmie en cas de myocardiopathie thalassémique par surcharge en fer.

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