|Year : 2015 | Volume
| Issue : 3 | Page : 267-269
Twiddler's syndrome revisited
Sumanto Mukhopadhyay, Joyanta Ghosh, Suraj Singh, Manish Vinayak, Chandan Misra, Dhurjati Prasad Sinha
Department of Cardiology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
|Date of Web Publication||23-Feb-2016|
Department of Cardiology, Institute of Post Graduate Medical Education and Research, Kolkata - 700 020, West Bengal
Source of Support: None, Conflict of Interest: None
Twiddler's Syndrome, first described by Bayliss, refers to a permanent malfunction of the pacemaker as a result of rotation of the device causing lead dislodgement. Since then, various cases of Twiddler and its variants such as Reel, Ratchet, and reverse Twiddler have been reported. This article reports two variants of Twiddler and reviews the literature on this interesting yet life-threatening complication of permanent pacing.
Keywords: Complication, Reel Syndrome, reverse Twiddler, Twiddler Syndrome
|How to cite this article:|
Mukhopadhyay S, Ghosh J, Singh S, Vinayak M, Misra C, Sinha DP. Twiddler's syndrome revisited. J Pract Cardiovasc Sci 2015;1:267-9
|How to cite this URL:|
Mukhopadhyay S, Ghosh J, Singh S, Vinayak M, Misra C, Sinha DP. Twiddler's syndrome revisited. J Pract Cardiovasc Sci [serial online] 2015 [cited 2019 Oct 16];1:267-9. Available from: http://www.j-pcs.org/text.asp?2015/1/3/267/177254
| Introduction|| |
Twiddler's Syndrome, first described by Bayliss, refers to a permanent malfunction of the pacemaker as a result of rotation of the device causing lead dislodgement.  Since then, various cases of Twiddler and its variants such as Reel, Ratchet, and reverse Twiddler have been reported. We report two variants of Twiddler and review the literature on this interesting complication.
| Case Reports|| |
A 62-year-old male presented with repeated episodes of syncope and 2:1 atrioventricular block on the electrocardiogram (ECG). The patient was advised to undergo a permanent pacemaker (PPM) implantation. On the day of PPM implantation, the patient was in sinus rhythm. A VVI pulse generator (Ventralite 940, Pacetronics) was implanted and connected to the ventricular lead. The lead was inserted with an introducer (PLI - 7F) into the right subclavian vein. The electrode (model - 3851 VB) with a bipolar configuration had passive fixation (tines) in the tip. The lead length was 56 cm. The stability of the lead tips was confirmed by dislodgement tests with excess loops of the lead. The intracardiac recordings from the ventricle disclosed adequate injury potentials and the pacing threshold was 0.5 V. The R wave was 11 mV. Lead impedance was 720 Ω. The lead was ligatured to the pectoral muscle with two separate nonabsorbable ligatures around suture sleeves. The wound was closed with absorbable suture material. On the 4 th postoperative day, the patient complained of intermittent jerky movements of the right upper abdomen. The movements were exacerbated and almost constantly present in the standing position. The ECG showed pacemaker spikes with failure to capture. However, there was no Stokes-Adams attack. Keeping the possibility of lead dislodgement, perforation, or malposition of the lead in mind, the patient was taken to the lab for fluoroscopy which revealed that the generator had rotated on its transverse axis rolling the lead around the generator and provoking a lead displacement [Figure 1]. It was repositioned.
A 64-year-old female with Stokes-Adams attack and complete heart block underwent VVI (St. Jude) pacing 1 year back presented with three episodes of syncope in the last 24 h. The patient was rushed to the emergency room where the ECG showed normal pacing complexes and the interrogation of the pacemaker revealed normal parameters. She was taken to the pacing lab where she had syncope and the ECG documented ventricular tachycardia (VT) with left bundle branch block morphology. On fluoroscopy, the lead loop around the pacemaker had been lost and was sagging in the right atrium and tricuspid annulus [Figure 2]. Her routine blood investigations and serum electrolytes were unremarkable. Coronary angiogram was normal with normal cardiac enzymes. The patient was started on amiodarone and revision of the pacemaker was done, and the patient stabilized with no further occurrence of VT.
| Discussion|| |
Twiddler's Syndrome refers to a permanent malfunction of the pacemaker as a result of rotation of the device causing lead dislodgement.  The reported incidence is 0.07-1.1%.  There are various mechanisms and risk factors proposed for the occurrence of the Twiddler's Syndrome. First, inadequate fixation of a transvenous lead at the site of introduction can be followed by dislocation.  Particular care must be taken to re-secure the lead if it becomes necessary to reposition the electrode tip within the right ventricular cavity. It is suggested that multiple ligatures of nonabsorbable material can be used at this site to provide greater stability. Second, a potential capstan mechanism is provided by pulse generators having a right angle plug between the lead and the pacer boss.  With this system, it is particularly important to prevent rotational movement of the pulse generator, since this results in traction on the lead. Theoretically, pacemakers with straight take-offs should permit rotation until lead fracture occurs, and the problem of traction is unlikely to arise. The right-angle take-off is a definite advantage and permits implantation of a more compact unit. Efforts should therefore be directed to the prevention of rotational movement of the pulse generator. Care must be taken to prevent enlargement of the subpectoral pocket.  There appears to be a natural tendency for fluid accumulation and pulse generator movement gradually to enlarge the pocket in the initial stages of healing. The unit should be implanted in the plane between the pectoralis major and minor muscles to avoid the loose subcutaneous tissue present in older patients. The pocket should be tight fit initially and should be plicated, if necessary, once the pulse generator is in position. Perhaps, most important is the use of a firm compression dressing for 5-7 days postoperatively.  In the case presented, only a light dressing was used and because of the movement of the inserted unit as well as fluid accumulation, this permitted enlargement of the initial dry pocket. The risk factors include elderly female patients with weak subcutaneous tissue, excess weight with subsequent weight loss, subcutaneous pocket, large pockets, and previous Twiddler's Syndrome, psychiatric disabilities, and dementia. ,,,
Reel's Syndrome is similar to Twiddler's Syndrome. In this case, the patient rotates the generator on its transverse axis (unlike the Twiddler's Syndrome where the lead turns around the pacemaker on its longitudinal axis), rolling the lead around the generator and provoking a lead displacement.  Chest radiography is crucial to diagnose this kind of complication. Ratchet syndrome can be defined as lead retraction and dislodgement due to progressive lead displacement through its fixation parts or protector sleeves, facilitated by movements of the ipsilateral arm and due to incomplete lead fixation to the protector sleeve, but without generator rotation over any of its axes. In this case, the problem could involve all system leads in a patient or, more commonly, only one of the leads, with all others in normal position; this is a key finding for identifying Ratchet syndrome and distinguishing it from the other two lead macro-dislodgement syndromes.  Recently, reverse Twiddler's Syndrome was described as pulse generator manipulation resulting in lead advancement rather than retraction. 
In the first case diagnosed as Reel's syndrome, the patient was a male and well built, weighed 68 kg at the first implantation as well as at the revision, and had not lost any weight. The device was implanted subcutaneously, and the pocket was not significantly larger than the pulse generator even after the revision. The patient denied any manipulation of the pulse generator. The wound looked outwardly normal. Thus, Twiddler's Syndrome can occur even when none of the accepted risk factors are present. The second case, a case of reverse Twiddler's Syndrome, was an elderly female with a properly implanted pacemaker without any history of weight loss and manipulation of the pulse generator presented with symptomatic VT due to irritation of the right ventricular inflow tract myocardium by the increased lead loop that prolapsed due to reverse Twiddler phenomenon. Both the cases were easily diagnosed with fluoroscopy and successfully treated by revising the pacemaker implantation. In both the cases, there was no stay suture of the pulse generator which could be an important risk factor for the rotation of the generator.
| Conclusion|| |
Twiddler's Syndrome is a potentially life-threatening condition usually occurring within the 1 st year of device implantation. Patients can be asymptomatic or present with diaphragmatic or brachial plexus stimulation or syncope or VT. Diagnosis can be readily achieved using chest X-ray or fluoroscopy. Expedient pocket and lead revision constitute the mainstay of therapy. Prevention of the syndrome can be achieved through suturing of the pulse generator to the fascia, submuscular implant, and use of active fixation leads. Patient education about the risks of pulse generator manipulation is of utmost importance.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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.
Hill PE. Complications of permanent transvenous cardiac pacing: A 14-year review of all transvenous pacemakers inserted at one community hospital. Pacing Clin Electrophysiol 1987;10 (3 Pt 1):564-70.
Khalilullah M, Khanna SK, Gupta U, Padmavati S. Pacemaker Twiddler′s Syndrome: A note on its mechanism. J Cardiovasc Surg (Torino) 1979;20:95-100.
Saliba BC, Ghantous AE, Schoenfeld MH, Marieb MA. Twiddler′s Syndrome with transvenous defibrillators in the pectoral region. Pacing Clin Electrophysiol 1999;22:1419-21.
Pereira PL, Trübenbach J, Farnsworth CT, Huppert PE, Claussen CD. Pacemaker and defibrillator Twiddler′s Syndrome. Eur J Radiol 1999;30:67-9.
Boyle NG, Anselme F, Monahan KM, Beswick P, Schuger CD, Zebede J, et al.
Twiddler′s Syndrome variants in ICD patients. Pacing Clin Electrophysiol 1998;21:2685-7.
Carnero-Varo A, Pérez-Paredes M, Ruiz-Ros JA, Giménez-Cervantes D, Martínez-Corbalán FR, Cubero-López T, et al.
"Reel Syndrome": A new form of Twiddler′s Syndrome? Circulation 1999;100:e45-6.
Cooper JM, Mountantonakis S, Robinson MR. Removing the twiddling stigma: Spontaneous lead retraction without patient manipulation. Europace 2010;12:1347-8.
Vlay SC. Reverse Twiddler′s Syndrome. Pacing Clin Electrophysiol 2009;32:146.
[Figure 1], [Figure 2]