Evaluation of cardiac status in iron-loaded thalassaemia patients following bone marrow transplantation: improvement in cardiac function during reduction in body iron burden

Iron-induced cardiac disease is the primary cause of death in transfused pa tients with thalassaemia major. The beneficial effects of deferoxamine mesy late on clinical cardiac disease have been well described but the impact of therapy on subclinical cardiac dysfunction is unknown. To assess the rever sibility of subclinical cardiac dysfunction we studied the cardiac status d uring iron depletion treatment (phlebotomy) in iron overloaded patients, cu red of thalassaemia by marrow transplantation, without clinical manifestati on of heart failure but with alteration in both left ventricular diastolic function and in contractility property. 32 patients were studied and demons trated a slight but significant impairment in the morphology and function i f compared with matched normal controls. 17 of these patients were submitte d to sequential echocardiographic evaluations during the phlebotomy program me. Following completion of the programme, normalization of the indices of contractility and normalization of diastolic function were observed, This s tudy indicates that transplanted thalassaemia patients with subclinical lef t ventricular diastolic dysfunction and impaired left ventricular contracti lity may reverse these processes with an effective regimen of iron reductio n such as phlebotomy.