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European Journal of Echocardiography 2003 4(4):331-333; doi:10.1016/S1525-2167(03)00013-1
© 2003 by European Society of Cardiology
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Copyright © 2003, The European Society of Cardiology

Dilated Cardiomyopathy in Friedreich's Ataxia: 2D Echo and Tissue-Doppler Analysis of Left Ventricular and Atrial Function

S.H Poulsen*, P Søgaard, J.E Nielsen-Kudsk and H Egeblad

Department of Cardiology, Aarhus University Hospital, Skejby Sygehus, Aarhus, Denmark

Received 2 December 2002; received in revised form 14 February 2003; accepted after revision 14 February 2003.

* Address correspondence to: Dr Steen Hvitfeldt Poulsen, Department of Cardiology, Skejby Hospital, Aarhus University Hospital, Aarhus N 8200, Denmark. Tel: +45-86495566; Fax: +45-89496009. steen.hvitfeldt@dadlnet.dk

Keywords: Friedreich's ataxia; dilated cardiomyopathy; tissue-Doppler

The first 10% of the full text of this article appears below.


   Introduction
 
Friedreich's ataxia (FA) is a hereditary spinocerebellar degenerative disease characterised clinically by ataxia, dysarthria, skeletal deformities, and progressive dystrophia of the skeletal muscles. The disease is frequently associated with concentric and, in some cases, eccentric hypertrophic cardiomyopathy. Presentation of a dilated cardiomyopathy with global dysfunction of the myocardium is rare and commonly supposed to represent an end-stage of a progressive transition from the hypertrophic cardiomyopathy[1]. We report the echocardiographic and tissue-Doppler findings in a case with FA who presented with dilated cardiomyopathy shortly after debut of cardiac symptoms.


   Case Report
 
. . . [Full Text of this Article]


   Discussion
 

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