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European Journal of Echocardiography Advance Access originally published online on December 17, 2008
European Journal of Echocardiography 2009 10(4):509-512; doi:10.1093/ejechocard/jen319
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Left ventricular non-compaction in identical twins with thalassaemia and cardiac iron overload

Matthew Luckie1,*, Bruce Irwin1, Satheesh Nair1, John Greenwood2 and Rajdeep Khattar1

1 Manchester Heart Centre, Manchester Royal Infirmary, Oxford Road, Manchester M13 9WL, UK
2 Leeds General Infirmary, Leeds, UK

Received 18 September 2008; accepted after revision 22 November 2008; online publish-ahead-of-print 17 December 2008.

* Corresponding author. Tel: +44 161 276 6576. E-mail address: mluckie{at}doctors.net.uk


  Abstract

Cardiac disease in patients with transfusion-dependent beta-thalassaemia major is well described. Cardiac manifestations may include left ventricular wall thickening and both systolic and diastolic dysfunctions. We describe a group of family members, including a pair of identical twins, each of whom suffered from thalassaemia major requiring multiple transfusions. Cardiac magnetic resonance demonstrated myocardial iron overload, and impairment of systolic function. Echocardiography confirmed both significant left ventricular systolic and diastolic impairment, along with features consistent with left ventricular non-compaction. This finding has not been noted in association with thalassaemia-related cardiac disease before. We then review the cardiac manifestations which occur in association with thalassaemia major.

Keywords: Left ventricular non-compaction; Thalassaemia major; Heart failure


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