Twiddlers Syndrome an Unusual Cause

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  • 8/6/2019 Twiddlers Syndrome an Unusual Cause


    Images In gerIatrIc cardIology 53tHe amerIcan JoUrnal oF gerIatrIc cardIology 2008 Vol. 17 no. 1

    A49-year-old patient presented with complaintsof uncomfortable, continuous pulsations ofthe upper abdomen and intermittent shocks in theright hemithorax when lying on her left side. A fewmonths prior, she had received cardiac resynchro-nization therapy (CRT) as treatment for an idio-pathic dilated cardiomyopathy (Figure 1). After aninitially marked improvement in functional class,she now reported a decrease in exercise capacity.There were no episodes of syncope.

    As the electrocardiogram showed improperpacemaker function, a chest x-ray was performed.This showed severe dislocation of both the atrialand coronary sinus (CS) leads in combination withincreased windings (Figure 2). The diagnosis oftwiddler's syndrome was made, despite the patientsdenial of external manipulation of the pacemaker.

    An invasive exploration was performed.Inspection of the pacemaker pocket showed thatthe suture fixating the pacemaker in the subcuta-neous intrafascial pocket was torn from the fascia.The leads were untangled (Figure 3), and theatrial lead was repositioned. However, an attemptto reposition the CS lead failed because of theinability to pass the S-shaped entrance of thelarge posterior branch with the over-the-wire CSlead, 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, result-

    ing in placement of a new epicardial left ventricularpacing lead via limited thoracotomy. In addition, anew subpectoral pacemaker pocket was created.

    As both the numbers of and indications forcardiac implantable devices increase, remarkable

    complications are more commonly encountered.1The term twiddler's syndrome refers to permanent

    malfunction of a pacemaker or other implanteddevices due to the patients manipulation of thepulse generator.2 Even though twiddler's syn-drome was first described nearly 40 years ago, itsprecise incidence remains unknown.3

    As cognitive impairment is common amongpatients with congestive heart failure, patientsreceiving CRT are likely to be at risk for develop-ing twiddler's syndrome.4 Nonetheless, this is thefirst report of a twiddler's syndrome variation witha biventricular pacing device. As the demand forCRT and therapy with an implantable cardioverterdefibrillator increases and the size of the devicesdeclines, twiddler's syndrome may prove to be anincreasingly common complication.

    Images in Geriatric Cardiologynvi c. n, md, si ei

    The University of Alabama at Birmingham, Birmingham, AL

    Twiddler's Syndrome: An Unusual Cause

    of Pacemaker Dysfunctionrb K. rizb, md; gb s. rui, mdFrom 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: ID: 5830Figure 1. Chest radiograph showing proper pacemaker

    and lead position.

  • 8/6/2019 Twiddlers Syndrome an Unusual Cause


    Images In gerIatrIc cardIology54 tHe amerIcan JoUrnal oF gerIatrIc cardIology 2008 Vol. 17 no. 1

    Some researchers advocate suturing the pace-maker firmly to the adjacent fascia after properreplacement of the pacing leads, in order to pre- vent future dislodgement by manipulation.5 Inaddition, subpectoral placement of the device willpresumably minimize this fiddling complication.

    Acknowledgement: The authors would like to thankBobSpringorum , cardiac technician, for his contribution tothe manuscript.


    1 Mond HG, Irwin M, Morillo C, et al. The world survey ofcardiac pacing and cardioverter defibrillators: calendaryear 2001.Pacing Clin Electrophysiol. 2004;27:955964.

    2 Nicholson WJ, Tuohy KA, Tilkemeier P. Twiddlers syn-drome.N Engl J Med. 2003;348:17261727.

    3 Bayliss CE, Beanlands DS, Baird RJ. The pacemaker-twid-dlers syndrome: a new complication of implantable trans- venous pacemakers.Can Med Assoc J. 1968;99:371373.

    4 Trojano L, Antonelli Incalzi R, Acanfora D, et al. Cognitive

    impairment: a key feature of congestive heart failure in theelderly.J Neurol. 2003;250:14561463.

    5 Tonino WA, Winter JB. The twiddler's syndrome.N Engl JMed. 2006;354:956.

    Figure 2. Chest radiograph showing dislodgement andtangled pacing leads.

    Figure 3. Twisted and tangled pacing leads in twiddler'ssyndrome.


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