HEART-TRANSPLANTATION FOR END-STAGE HEART-FAILURE CAUSED BY IRON OVERLOAD

Few reports exist concerning heart transplantation in recipients with end-stage myocardiopathy-associated heart failure caused by iron overl oad occurring with beta-thalassaemia, Diamond-Blackfan syndrome or hae mochromatosis. Seven potential transplant candidates (six male, one fe male, mean age 26 years) with such heart failure, following desferriox amine application subcutaneously over a number of years, and intraveno usly during their hospitalization before transplantation, were retrosp ectively analysed. Five were New York Heart Association (NYHA) class I V, three experienced one or more resuscitations immediately before tra nsplantation could be performed. Continuous, high-volume, veno-venous haemofiltration was necessary in two patients. One of these two candid ates additionally had to be bridged, first with a right ventricular, t hen with a biventricular assist device. Five of the seven patients sur vived, two with haemochromatosis, one with beta-thalassaemia major and one with Diamond-Blackfan syndrome following transplantation. One non transplanted candidate with beta-thalassaemia major has been recompens ated for 5 years. Survival was 14-74 months. Our results demonstrate t he feasibility and indication of transplantation in patients with such heart failure and the satisfying outcome of immunosuppression is desc ribed.