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Heart transplantation for end-stage heart failure caused by iron overload
Authors:Michael M.,Koerner ,Gero,Tenderich ,Kazutomo,Minami ,Edzard,zu Knyphausen ,Hermann,Mannebach ,Knut,Kleesiek ,Hans,Meyer &   Reiner,Koerfer
Affiliation:Departments of Thoracic and Cardiovascular Surgery; Paediatric Cardiology; Cardiology; Institute of Laboratory and Transfusion Medicine, Heart Centre Northrhine-Westphalia, Ruhr University of Bochum, Bad Oeynhausen, Germany
Abstract:Few reports exist concerning heart transplantation in recipients with end-stage myocardiopathy-associated heart failure caused by iron overload occurring with β-thalassaemia, Diamond-Blackfan syndrome or haemochromatosis. Seven potential transplant candidates (six male, one female, mean age 26 years) with such heart failure, following desferrioxamine application subcutaneously over a number of years, and intravenously during their hospitalization before transplantation, were retrospectively analysed. Five were New York Heart Association (NYHA) class IV, three experienced one or more resuscitations immediately before transplantation could be performed. Continuous, high-volume, veno-venous haemofiltration was necessary in two patients. One of these two candidates additionally had to be bridged, first with a right ventricular, then with a biventricular assist device. Five of the seven patients survived, two with haemochromatosis, one with β-thalassaemia major and one with Diamond-Blackfan syndrome following transplantation. One non-transplanted candidate with β-thalassaemia major has been recompensated for 5 years. Survival was 14–74 months. Our results demonstrate the feasibility and indication of transplantation in patients with such heart failure and the satisfying outcome of immunosuppression is described.
Keywords:heart transplantation    β-thalassaemia    Diamond-Blackfan syndrome    haemochromatosis    immunosuppression
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