Volume 15, Number 1, January-February 2008

Chronic transfusion, iron overload and cardiac dysfunction: a multi-dimensional perspective
Hussain Isma’eel, Maria D Cappellini, Ali Taher

Cardiac dysfunction is common in patients with thalassaemia and is the leading cause of mortality in adult patients. Transfusional iron overload can affect heart function by directly damaging tissue through iron deposition or via ironmediated effects at other sites. The main cardiac abnormalities reported in patients with thalassaemia and iron overload are left ventricular systolic and diastolic dysfunction, pulmonary hypertension, valvulopathies, arrhythmias and pericarditis. Prevalence varies according to the type of thalassaemia. However, even though patients with thalassaemia intermedia require fewer transfusions than those with thalassaemia major, they are still at high risk for cardiac complications. With the introduction of new technologies such as cardiac magnetic resonance T2*, the early detection of cardiac iron overload and associated cardiac dysfunction is now possible, allowing time for reversal through iron chelation therapy. Although chelation therapy can reverse ironmediated cardiac disease by removing iron from iron-loaded cardiomyocytes and by alleviating the systemic iron overload contributing to heart failure, the challenges of deferoxamine infusions can significantly impact on compliance and, therefore, prognosis. The introduction of new oral iron chelators, together with improved understanding of the mechanisms and consequences of transfusional iron overload, should allow the continued improvement in cardiac outcomes for patients with thalassaemia and other transfusion-dependent anaemias.

Br J Cardiol 2008;15:40-45.

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