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  • Early detection of left ventricular dysfunction in patients with β thalassaemia major by dobutamine stress echocardiography

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Early detection of left ventricular dysfunction in patients with β thalassaemia major by dobutamine stress echocardiography
  1. L Hui1,
  2. M P Leung1,
  3. S Y Ha2,
  4. A K T Chau1,
  5. Y F Cheung1
  1. 1Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Grantham Hospital, The University of Hong Kong, Hong Kong China
  2. 2Division of Paediatric Haematology and Oncology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong
  1. Correspondence to:
    Dr L Hui, Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Grantham Hospital, The University of Hong Kong, 125 Wong Chuk Hang Road, Hong Kong, China;
    lhui{at}hkucc.hku.hk

https://doi.org/10.1136/heart.89.6.669

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    Myocardial iron deposition, among other factors, contributes to left ventricular (LV) dysfunction in patients with β thalassaemia major.1 Nonetheless, conventional indices have not been particularly useful for its early detection. Importantly, cardiac function deteriorates rapidly once clinical symptoms develop. Dobutamine stress echocardiography (DSE) is well accepted in the evaluation of myocardial dysfunction caused by coronary artery disease in adults.2 In children, DSE has been used for early detection of LV dysfunction in long term childhood cancer survivors.3 In this study, we assessed the usefulness of DSE for early detection of LV dysfunction in thalassaemia patients.

    METHODS

    Twenty six patients (17 females) with β thalassaemia major with a mean (SD) age of 17.4 (8.3) years (range 4–33 years) were studied. Their body surface area (BSA) was 1.12 (0.20) m2. None had congestive heart failure clinically and all were in New York Heart Association (NYHA) functional class I at initial assessment. Monthly blood transfusion was started at a median of 11 months (range 3–96 months), while desferrioxamine was started at a median age of 30 months (range 25–114 months). Post-transfusion haemoglobin was targeted at 14 g/dl (8.7 mmol/l), and echocardiographic studies were performed within one week of transfusion to minimise confounding influence of anaemia. Only one of the patients had undergone splenectomy. Twenty healthy subjects (13 females) aged 10.4 (4.5) years (range 4–15 years), …

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