Antioxidant Therapy for Severe Cardiac Failure Induced by Iron Overload Secondary to Dyserythropoietic Anaemia

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 augme...

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Bibliographic Details
Published in:Heart, lung & circulation lung & circulation, 2007-10, Vol.16 (5), p.394-397
Main Authors: van der Merwe, Juliana, MPhil, Leong, Jee-Yoong, MBBS, Kaye, David, FRACP, Opat, Stephen, FRACP, Bergin, Peter J., FRACP, Taylor, Andrew J., FRACP, Rosenfeldt, Franklin, FRACP
Format: Article
Language:English
Subjects:
Anemia, Dyserythropoietic, Congenital - complications
Anemia, Dyserythropoietic, Congenital - drug therapy
Antioxidants - therapeutic use
Cardiac metabolism
Cardiovascular
Child
Coenzyme Q 10
Coenzymes - blood
Congenital dyserythropoietic anaemia
Drug Therapy, Combination
Female
Ferritins - blood
Heart failure
Heart Failure - drug therapy
Heart Failure - etiology
Humans
Iron Chelating Agents - therapeutic use
Lipid Peroxidation - drug effects
Severity of Illness Index
Ubiquinone - analogs & derivatives
Ubiquinone - blood
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Summary: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.
ISSN:1443-9506
1444-2892
DOI:10.1016/j.hlc.2006.12.010