Case Presentation
A 55-year-old male patient presents with frequent pacemaker firing.
Figure 1. X-ray of presenting patient.[1]
1. What does the x-ray show?
2. What can you tell from x-ray imaging of cardiac devices?
3. What is the management?
Twiddler Syndrome
Pictured is a biventricular, left-sided pacemaker demonstrating lead dislodgement due to Twiddler Syndrome. Twiddler Syndrome is a pacemaker complication caused by external manipulation of the pacemaker box, typically by the patient. Patients with dementia seem to be at the highest risk for Twiddler Syndrome due to their propensity toward “lint-picking” behavior or carphologia.[1,2]
In this biventricular pacemaker, the three leads should be situated in the left ventricle, right ventricle, and right atrium. In the image presented, there is migration of the distal leads (blue and red arrows) such that one lead projects over the right ventricle (red arrow), one lead over the superior vena cava (light blue arrow), and one lead over the left brachiocephalic vein (dark blue arrow). Further, there is looping of the proximal leads next to the pulse generator (black arrow) from external pacemaker manipulation.
Figure 2. Migration of leads as described above.[1]
Brief Review of the Radiology of Pacemakers
The two types of cardiac conduction devices (CCD) are pacemakers and implantable cardioverter-defibrillators (ICD). Pacemakers are further broken down into three groups: single-chamber pacemaker (typically used to treat dysrhythmias in which the conduction pathways are still intact); dual-chamber pacemaker (typically used to treat dysrhythmias by coordinating the atrium and ventricle); and biventricular pacemaker (typically used in severe systolic dysfunction to synchronize the left and right ventricles).
Figure 3. Simplified table of pacemaker types. Original figure.
Pacemakers and ICDs can be combined to produce a combination CCD. Combination CCDs are mainly employed in the treatment of patients with severe CHF (EF < 30%), as these patients are at a higher risk of developing fatal cardiac tachydysrhythmias, such as ventricular fibrillation or ventricular tachycardia. In a combination CCD, one of the leads will have a shock coil located in the SVC-atrial junction and one in the ventricle. Shock coils appear as a metallic density along the lead and are typically much longer than one would expect of the lead tip, which appears as a coil or prong (depending on the fixation mechanism).
Figure 4. AP and lateral chest x-ray of a combination pacemaker and ICD with adequate lead placement.[3]
Figure 5. Correlation between actual lead tip (A) and x-ray representation (B).[4]
Figure 6. Normal cardiac anatomy overlaying a frontal and lateral chest x-ray.[4]
Common Patient Presentations
A. Right arm twitching
Figure 7. Lead initially placed in the right ventricle, now displaced to the SVC and likely stimulating the brachial plexus. There is also proximal lead looping around the pulse generator.[5]
Figure 8. (A) Biventricular pacemaker with complete dislodgement of the right ventricular lead and mild dislodgement of the left ventricular lead, likely stimulating the diaphragm via the phrenic nerve. (B) Severely tangled proximal wires in the same patient.[6]
C. Defibrillator shocks every 5-15 min
Upon further history, the patient has been complaining of pruritus at the site of pacemaker implantation since implantation.
Figure 9. Right atrial lead dislodged to the SVC junction and right ventricular lead overlying the right atrium. There is twisting of the wires near the pulse generator.[7]
Figure 10. This patient had dislodgement of her right ventricular lead causing intermittent loss of capture.[8] Device failure is the most common presentation of all, such that patients will present with the symptoms which historically necessitated the index device implantation.
Management
Twiddler’s syndrome always requires revision. Typically, these patients will have new leads placed, or if old leads are unable to be recovered, the pacemaker is de-activated and a new pacemaker is placed. The pacemaker is secured with sutures and the pacemaker pocket is typically decreased in size to allow less movement of the device.[3]
More Resources
References
1. Harel G, Georgeta E, Copperman Y. Twiddler’s syndrome: a rare cause of pacemaker failure. Isr Med Assoc J. 2008;10(2):160-161.
2. Castillo R, Cavusoglu E. Twiddler’s syndrome: an interesting cause of pacemaker failure. Cardiology. 2006;105(2):119-121. doi:10.1159/000090213
3. Schulman PM, Rozner MA, Sera V, Stecker EC. Patients with pacemaker or implantable cardioverter-defibrillator. Med Clin North Am. 2013;97(6):1051-1075. doi:10.1016/j.mcna.2013.05.004
4. Aguilera AL, Volokhina YV, Fisher KL. Radiography of cardiac conduction devices: a comprehensive review. Radiographics. 2011;31(6):1669-1682. doi:10.1148/rg.316115529
5. Davis BP. Images in emergency medicine: An elderly woman with arm twitching. Ann Emerg Med. 2009;54(3):476-483. doi:10.1016/j.annemergmed.2009.02.014
6. Riezebos RK, de Ruiter GS. Twiddler’s syndrome: an unusual cause of pacemaker dysfunction. Am J Geriatr Cardiol. 2008;17(1):53-54. doi:10.1111/j.1076-7460.2007.05830.x
7. Gallahue FE, Walker A. Twiddler’s syndrome: The slow twist of a pruritic pacemaker. J Am Coll Emerg Physicians Open. 2020;1(4):674-675. Published 2020 Jun 15. doi:10.1002/emp2.12148
8. Tahirovic E, Haxhibeqiri-Karabdic I. Twiddler’s Syndrome: Case Report and Literature Review. Heart Views. 2018;19(1):27-31. doi:10.4103/HEARTVIEWS.HEARTVIEWS_89_17
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