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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 3  |  Page : 118-121  

Twiddler's syndrome: An unusual cause of repeated shocks by implantable cardioverter-defibrillator in an asymptomatic patient


1 Department of Internal Medicine, Cleveland Clinic/Akron General Hospital, Akron, OH, USA
2 Department of Cardiovascular Medicine, Cleveland Clinic/Akron General Hospital, Akron, OH, USA
3 Department of Heart and Vascular Center, Case Western Reserve University/MetroHealth Medical Center, Cleveland, OH, USA

Date of Web Publication26-Sep-2019

Correspondence Address:
Dr. Ahmad Jabri
Department of Internal Medicine, Akron General Cleveland Clinic, Akron, Ohio
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HEARTVIEWS.HEARTVIEWS_45_19

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   Abstract 


Twiddler's syndrome refers to malfunction of pacemaker or implantable cardioverter-defibrillator (ICD) resulting from deliberate or unintentional twisting of pulse generator in device pocket, resulting in lead dislodgment. We report a case of a 65-year-old woman with multiple ICD shocks and antitachycardia pacing events on device interrogation, while she was unaware of the ICD discharges. Twiddler's syndrome is rare and mostly occurs in obese or elderly because of loose subcutaneous tissue allowing pulse generator to rotate in its pocket. It can be prevented by patient education against hand manipulating the generator. Smaller device pocket or suturing generator to fascia can also prevent future episodes.

Keywords: Implantable cardioverter-defibrillator (ICD), shock, twiddler's syndrome


How to cite this article:
Jabri A, Laiq Z, Nabeel Y. Twiddler's syndrome: An unusual cause of repeated shocks by implantable cardioverter-defibrillator in an asymptomatic patient. Heart Views 2019;20:118-21

How to cite this URL:
Jabri A, Laiq Z, Nabeel Y. Twiddler's syndrome: An unusual cause of repeated shocks by implantable cardioverter-defibrillator in an asymptomatic patient. Heart Views [serial online] 2019 [cited 2019 Oct 15];20:118-21. Available from: http://www.heartviews.org/text.asp?2019/20/3/118/267844




   Introduction Top


Twiddler's syndrome occurs as a result of pacemaker or implantable cardioverter-defibrillator (ICD) pulse generator twisting, resulting in the loss of device function. Therefore, twiddler's syndrome should be recognized and corrected early. It may be asymptomatic or may present with a wide spectrum of symptoms, depending on the site of the lead dislodgment.


   Case Presentaton Top


We report a case of a 65-year-old woman with medical history of heart failure with reduced ejection fraction, hypertension, and diabetes mellitus type II who had multiple ICD shocks and anti-tachycardia pacing events on device interrogation, while she was unaware of the ICD discharges. The patient denied shortness of breath or chest pain. She had a history of ventricular fibrillation arrest in the past and had undergone placement of an ICD 7 months ago. Device interrogation revealed lead sensing and captured failure with drop-in shock impedance. Electrogram showed lead noise misinterpreted as ventricular fibrillation by the device, resulting in inappropriate ICD shocks [Figure 1], [Figure 2], [Figure 3], [Figure 4]. Chest X-ray revealed that ICD lead had dislodged from the right ventricle (RV), retracted into axillary vein, and coiled around the generator while generator itself was flipped around its axis [Figure 5], [Figure 6], [Figure 7]. She underwent lead revision with extraction of the dislodged lead and insertion of new RV lead.
Figure 1: Electrogram from implantable cardioverter-defibrillator showing noise being interpreted as ventricular fibrillation

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Figure 2: Electrogram from implantable cardioverter-defibrillator showing attempted antitachycardia pacing

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Figure 3: Electrogram from implantable cardioverter-defibrillator showing implantable cardioverter defibrillator shock

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Figure 4: Electrogram from implantable cardioverter-defibrillator showing attempt at postshock pacing

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Figure 5: Chest X-ray from the time of initial implantable cardioverter-defibrillator implantation with correct lead position in right ventricle

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Figure 6: Chest X-ray at the time of presentation with lead retracted into axillary vein and coiled around the generator while the generator itself flipped on its axis

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Figure 7: Magnified image of Figure 6

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   Discussion Top


First introduced in 1968, twiddler's syndrome refers to malfunction of pacemaker or ICD, resulting from deliberate or unintentional twisting of pulse generator in device pocket resulting in lead dislodgment.[1] The incidence of twiddler's syndrome lies between 0.07% and 7%.[2] Twiddler's syndrome mostly occurs in obese or elderly because of loose subcutaneous tissue allowing pulse generator to rotate in its pocket. Other risk factors include female gender, weight loss, upper limb exaggerated movements, device manipulation, or large pocket size relative to the defibrillator.[3],[4],[5]

Most cases occur in the 1st year after implantation of the defibrillator;[6],[7] however, twiddler's syndrome can still occur after that.[8] The established guidelines for ICD placement follow-up are recommended after 72 h from placement and then after 2–12 weeks. Thereafter, in-person or remote follow-ups in 3–6 months can be performed.[9] These follow-up intervals are assigned to identify dysfunctioning ICDs. Twiddler's syndrome can be asymptomatic [8] or represented with various symptoms depending on the final site of the displaced lead. Most symptoms occur due to missed capture, stimulation of nearby structures, or improper exaggerated response to an input.[10]

Twiddler's syndrome can be life-threatening due to ventricular arrhythmias and usually requires rapid intervention with lead revision. A plain chest X-ray can quickly identify lead dislodgment and twiddling of pulse generator. To best aid in diagnosis of twiddler's syndrome, an electrocardiogram, chest X-ray, and pacemaker interrogation should be obtained as routine workup.[11] To restore the ICD function, correction of lead dislodgment, new lead installation, or pulse generator relocation can also be considered.[10] Anchoring sutures to the pectoralis fascia can be performed to prevent the re-twisting of the generator or leads. Another approach described in the literature to prevent the dislodgment of the lead was nonabsorbable antimicrobial pouches.[12] In the study by Osoro et al., the rate of twiddler's syndrome recurrence with nonabsorbable antimicrobial pouches used in correction of lead dislodgment was compared to other approaches, including sutures to the underlying pectoralis fascia.[12] The results showed a rate of 0% recurrence for the antimicrobial pouches compared to 50% recurrence to other approaches, with a P < 0.05.[12]

Patient education against hand manipulating the generator is another major determinator for twiddler's syndrome recurrence, and therefore, concealing should be provided. Patient selection may also play a role in recurrent cases of twiddler's syndrome. Patients with mental disorders or with high potential for psychoorganic syndrome will tend to disregard the manipulation instructions.[11] Moreover, appropriate follow-up visits should be instructed to patients to aid in diagnosis of missed asymptomatic cases like our case. More frequent device interrogation sessions may be performed in the high-risk population.


   Conclusion Top


Twiddler's syndrome is a vital lethal diagnosis that necessitates early corrective measures. Diagnosis requires high level of suspicion due to the variety of presentations including asymptomatic patients to others with random complaints related to the site of the device dislodgment. Proper device interrogation visits should be advised to patients for better detection.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Bayliss CE, Beanlands DS, Baird RJ. The pacemaker-twiddler's syndrome: A new complication of implantable transvenous pacemakers. Can Med Assoc J 1968;99:371-3.  Back to cited text no. 1
    
2.
Fahraeus T, Höijer CJ. Early pacemaker twiddler syndrome. Europace 2003;5:279-81.  Back to cited text no. 2
    
3.
Veltri EP, Mower MM, Reid PR. Twiddler's syndrome: A new twist. Pacing Clin Electrophysiol 1984;7:1004-9.  Back to cited text no. 3
    
4.
Sidhu GS, Seifi A, Zangiabadi AH, Reinig M. Follow your leads: A case of twiddler's syndrome. South Med J 2009;102:871-2.  Back to cited text no. 4
    
5.
Nicholson WJ, Tuohy KA, Tilkemeier P. Twiddler's syndrome. N Engl J Med 2003;348:1726-7.  Back to cited text no. 5
    
6.
Sharifi M, Inbar S, Neckels B, Shook H. Twiddling to the extreme: Development of twiddler syndrome in an implanted cardioverter-defibrilllator. J Invasive Cardiol 2005;17:195-6.  Back to cited text no. 6
    
7.
DeMarco DC, Xuereb RG. 'Twiddling' of the pacemaker resulting in lead dislodgement. Malta Med J 2009;21:38-41.  Back to cited text no. 7
    
8.
Parikh V, Barsoum EA, Morcus R, Azab B, Lafferty J, Kohn J. Unique presentation of twiddler's syndrome. World J Cardiol 2013;5:207-9.  Back to cited text no. 8
    
9.
Wilkoff BL, Auricchio A, Brugada J, Cowie M, Ellenbogen KA, Gillis AM, et al. HRS/EHRA expert consensus on the monitoring of cardiovascular implantable electronic devices (CIEDs): Description of techniques, indications, personnel, frequency and ethical considerations: Developed in partnership with the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA); and in collaboration with the American College of Cardiology (ACC), the American Heart Association (AHA), the European Society of Cardiology (ESC), the Heart Failure Association of ESC (HFA), and the Heart Failure Society of America (HFSA). Endorsed by the Heart Rhythm Society, the European Heart Rhythm Association (a registered branch of the ESC), the American College of Cardiology, the American Heart Association. Europace 2008;10:707-25.  Back to cited text no. 9
    
10.
Bozyel S, Aksu T, Erdem Guler T, Serhan Ozcan K, Aktas M. Pectoral muscular twitching: A rare manifestation of spontaneous twiddler syndrome. J Geriatr Cardiol 2017;14:532-3.  Back to cited text no. 10
    
11.
Tahirovic E, Haxhibeqiri-Karabdic I. Twiddler's syndrome: Case report and literature review. Heart Views 2018;19:27-31.  Back to cited text no. 11
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12.
Osoro M, Lorson W, Hirsh JB, Mahlow WJ. Use of an antimicrobial pouch/envelope in the treatment of twiddler's syndrome. Pacing Clin Electrophysiol 2018;41:136-42.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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