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Extreme pacemaker reel syndrome in an elderly patient with cognitive impairment

26 March 2020
Volume 29 · Issue 6

Abstract

Twiddler's syndrome is a rare cause of pacemaker failure, where patient manipulation of the pulse generator results in lead dislodgement or retraction. Variations in manifestation have been identified including reel syndrome, where rotation occurs around the transverse axis resulting in coiling of the leads, and ratchet syndrome where arm movement results in lead displacement. Device manipulation leading to device failure has been documented in up to 1.7% of implants, particularly in patients with large pockets or mental disorders. Such complications have serious consequences, particularly in pacing-dependent patients where loss of capture may result in asystole. This article reviews the case of an 84-year-old patient presenting at 8-month pacemaker follow-up in complete heart block with no evidence of pacemaker function.

Twiddler's syndrome, first described by Bayliss et al (1968), is a rare cause of pacemaker failure, where patients manipulate the pulse generator resulting in lead dislodgement or retraction. Characterised by rotation of the device (Figure 1), this action results in a twisting of the leads, leading to lead dislodgement and cessation of pacing, and can often cause lead fracture or insulation failure (Abrams et al, 1995; Pereira et al, 1999; Bali et al, 2013). Variations in manifestation have been identified, including reel syndrome, where rotation occurs around the transverse axis resulting in a coiling of the leads around the device (Carnero-Varo et al, 1999), and ratchet syndrome where arm movement results in lead displacement (Arias et al, 2012). Device manipulation leading to device failure has been documented in 0.07-1.7% of implants (Fahraeus and Höijer, 2003; Balaschak, 2012) with a higher incidence in patients who have large pacemaker pockets, or in those who have psychiatric disorders or mental health problems (Castillo and Cavusoglu, 2006; Barold and Stroobandt, 2009). Such complications have serious consequences, particularly in pacing-dependent patients where loss of capture may result in asystole.

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