A case report: Iron overload in transfusion dependent thalassaemia with cardiomyopathy and arrhythmias

CCK Liam Liam, YL Boo, SM Lim, SG Toh

Abstract

Cardiac dysfunction is a common complication and a leading cause of mortality in patients with thalassaemia. Iron overload affects heart function by directly damaging tissue through iron deposition or iron-mediated inflammatory effects and endocrinopathies. Case report: Our patient is a 24-year-old man with beta thalassaemia major, on stable transfusion requirements with pre-transfusion hemoglobin (Hb) of 8-10g/dL. His ferritin increased to 8021μg/L due to non-compliance to iron chelators. He started being symptomatic of heart failure in early 2016 and was subsequently admitted with atrial fibrillation and decompensated heart failure.  His magnetic resonance imaging (MRI) T2* revealed severe cardiac iron overload with an ejection fraction (EF) of 35-40%, and very severe liver iron concentration (LIC) of 52.4 mg/g dry weight. He was given intravenous infusion desferrioxamine and deferiprone together with anti arrhythmic and heart failure therapies. His symptoms improved and ferritin decreased drastically within 6 months. However, his repeated MRI T2* showed worsening cardiac iron loading despite improvements in LIC. He progressed to end stage heart failure and subsequently succumbed to his illness. Discussion: Patients with thalassaemia are at higher risk for cardiac complications. The use of MRI T2* and ferritin to detect cardiac iron overload and its associated subclinical cardiac dysfunction at an early stage will allow early initiation of iron chelation. Our case highlights the importance of good iron chelation to prevent the onset of cardiomyopathy as it may be difficult to reverse once it has set in.

 

    

Keywords: iron overload, thalassaemia, cardiomyopathy

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