AI Article Synopsis
- The study focuses on heart transplantation in seven young patients suffering from heart failure due to iron overload related to conditions like beta-thalassemia, Diamond-Blackfan syndrome, and hemochromatosis.
- Most patients were in advanced heart failure (NYHA class IV) and required intensive treatments, including mechanical support, before undergoing transplantation.
- Out of the seven candidates, five survived post-transplant, showing that heart transplantation can be a viable option for those affected by these unique conditions.
Article Abstract
Few reports exist concerning heart transplantation in recipients with end-stage myocardiopathy-associated heart failure caused by iron overload occurring with beta-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 beta-thalassaemia major and one with Diamond-Blackfan syndrome following transplantation. One non-transplanted candidate with beta-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.
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| http://dx.doi.org/10.1046/j.1365-2141.1997.482693.x | DOI Listing |
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