We present a case of a patient with longstanding transfusion-dependent congenital dyserythropoietic anaemia (CDA) who developed cardiomyopathy despite iron chelation therapy. She presented with severe heart failure that responded poorly to conventional therapy, recovering only when therapy was augmented with metabolic agents including antioxidants and with increased iron chelation. The present case gives support to the concept of treating oxidatively induced heart failure with metabolic and antioxidant therapy. This therapy may have wider application in refractory heart failure and in the prevention of cardiomyopathy in patients receiving regular red cell transfusions who are at risk of transfusional haemosiderosis.
- Cardiac metabolism
- Coenzyme Q
- Congenital dyserythropoietic anaemia
- Heart failure