Images in Geriatric Cardiology

navin c. nanda, md, section editor The University of Alabama at Birmingham, Birmingham, AL

Twiddler's Syndrome: An Unusual Cause of Pacemaker Dysfunction

robert K. riezebos, md; gysbert s. de ruiter, md
From the Department of Cardiology, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands Address for correspondence: R.K. Riezebos, Department of Cardiology, Onze Lieve Vrouwe Gasthuis, Oosterpark 9, 1091AC Amsterdam, the Netherlands E-mail: r.k.riezebos@xs4all.nl

49-year-old patient presented with complaints of uncomfortable, continuous pulsations of the upper abdomen and intermittent shocks in the right hemithorax when lying on her left side. A few months prior, she had received cardiac resynchronization therapy (CRT) as treatment for an idiopathic dilated cardiomyopathy (Figure 1). After an initially marked improvement in functional class, she now reported a decrease in exercise capacity. There were no episodes of syncope. As the electrocardiogram showed improper pacemaker function, a chest x-ray was performed. This showed severe dislocation of both the atrial and coronary sinus (CS) leads in combination with increased windings (Figure 2). The diagnosis of twiddler's syndrome was made, despite the patients denial of external manipulation of the pacemaker. An invasive exploration was performed. Inspection of the pacemaker pocket showed that the suture fixating the pacemaker in the subcutaneous intrafascial pocket was torn from the fascia. The leads were untangled (Figure 3), and the atrial lead was repositioned. However, an attempt to reposition the CS lead failed because of the inability to pass the S-shaped entrance of the large posterior branch with the over-the-wire CS lead, presumably as a result of fibrosis of the vein caused by mild dissection at initial implantation. Another suitable CS branch was not available. The options were relayed to the patient, resulting in placement of a new epicardial left ventricular pacing lead via limited thoracotomy. In addition, a new subpectoral pacemaker pocket was created. As both the numbers of and indications for cardiac implantable devices increase, remarkable

complications are more commonly encountered.1 The term twiddler's syndrome refers to permanent malfunction of a pacemaker or other implanted devices due to the patients manipulation of the pulse generator.2 Even though twiddler's syndrome was first described nearly 40 years ago, its precise incidence remains unknown.3 As cognitive impairment is common among patients with congestive heart failure, patients receiving CRT are likely to be at risk for developing twiddler's syndrome.4 Nonetheless, this is the first report of a twiddler's syndrome variation with a biventricular pacing device. As the demand for CRT and therapy with an implantable cardioverter defibrillator increases and the size of the devices declines, twiddler's syndrome may prove to be an increasingly common complication.

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Figure 1. Chest radiograph showing proper pacemaker and lead position.

Images In gerIatrIc cardIology

tHe amerIcan JoUrnal oF gerIatrIc cardIology 2008 Vol. 17 no. 1

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The American Journal of Geriatric Cardiology (ISSN 1076-7460) is published bimonthly (Jan., March, May, July, Sept., Nov.) by Le Jacq, a Blackwell Publishing imprint, located at Three Enterprise Drive, Suite 401, Shelton, CT 06484. Copyright 2007 by Le Jacq. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording, or any information storage and retrieval system, without permission in writing from the publishers. The opinions and ideas expressed in this publication are those of the authors and do not necessarily reflect those of the Editors or Publisher. For copies in excess of 25 or for commercial purposes, please contact Ben Harkinson at BHarkinson@bos.blackwellpublishing.com or 781-388-8511.

Some researchers advocate suturing the pacemaker firmly to the adjacent fascia after proper replacement of the pacing leads, in order to prevent future dislodgement by manipulation.5 In addition, subpectoral placement of the device will presumably minimize this fiddling complication.
Acknowledgement: The authors would like to thank Bob Springorum, cardiac technician, for his contribution to the manuscript.

RefeRences
1 Mond HG, Irwin M, Morillo C, et al. The world survey of 2 Figure 2. Chest radiograph showing dislodgement and tangled pacing leads. 3 4 5
cardiac pacing and cardioverter defibrillators: calendar year 2001. Pacing Clin Electrophysiol. 2004;27:955964. Nicholson WJ, Tuohy KA, Tilkemeier P. Twiddlers syndrome. N Engl J Med. 2003;348:17261727. Bayliss CE, Beanlands DS, Baird RJ. The pacemaker-twiddlers syndrome: a new complication of implantable transvenous pacemakers. Can Med Assoc J. 1968;99:371373. Trojano L, Antonelli Incalzi R, Acanfora D, et al. Cognitive impairment: a key feature of congestive heart failure in the elderly. J Neurol. 2003;250:14561463. Tonino WA, Winter JB. The twiddler's syndrome. N Engl J Med. 2006;354:956.

Figure 3. Twisted and tangled pacing leads in twiddler's syndrome.

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Images In gerIatrIc cardIology

tHe amerIcan JoUrnal oF gerIatrIc cardIology 2008 Vol. 17 no. 1

The American Journal of Geriatric Cardiology (ISSN 1076-7460) is published bimonthly (Jan., March, May, July, Sept., Nov.) by Le Jacq, a Blackwell Publishing imprint, located at Three Enterprise Drive, Suite 401, Shelton, CT 06484. Copyright 2007 by Le Jacq. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording, or any information storage and retrieval system, without permission in writing from the publishers. The opinions and ideas expressed in this publication are those of the authors and do not necessarily reflect those of the Editors or Publisher. For copies in excess of 25 or for commercial purposes, please contact Ben Harkinson at BHarkinson@bos.blackwellpublishing.com or 781-388-8511.