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.
According to the Office for National Statistics the UK population is getting older (Office for National Statistics, 2019), and with this ageing population device implants have shown a steady rise since 2004; however, the number of first pacemaker implants has remained static for England for the past 2 reported years, with figures for 2015/16 and 2016/17 for England alone showing 555 pacemaker implants per million population (Cunningham et al, 2017; National Cardiac Audit Programme, 2019). It has been estimated that patients over the age of 75 years account for over half of pacemaker implants (Armaganijan et al, 2012) and these individuals are more likely to have confounding comorbidities. Due to this, it is therefore probable that complications enhanced by patient factors will become a more common occurrence, meaning that correct long-term device management is important.
Case study
A wheelchair-bound 82-year-old female with documented dementia presented to the emergency department (ED) following syncopal episodes. Eight months earlier a single-chamber VVI pacemaker had been implanted for third-degree atrioventricular (AV) block. A Biotronik Effecta SR VVI(R) with a passive fixation bipolar ventricular lead had been implanted via the left subclavian approach. However, the patient had failed to attend scheduled follow-up clinics since her 1-week check.
On presentation in A&E an electrocardiogram (ECG) showed third-degree AV block with a ventricular rate of 30-35 beats per minute (bpm) and no evidence of pacemaker function (Figure 1). Device interrogation revealed normal lead impedance (781 Ω), however, device tests showed total loss of capture and sensing. The patient was sent for a chest X-ray with radiographs revealing the cause of failure to be reel syndrome, because the lead was totally retracted and tightly coiled close to the generator (Figure 2). Device manipulation by the patient was observed during follow-up and because of the patient's dementia it was e vident that it would be difficult to prevent this behaviour. Following consultation, a right-sided approach was selected for a new device implant, with the old device left in situ. A replacement device of the same type and passive bipolar ventricular lead were successfully implanted and programmed to VVI; rate responsiveness was deemed unnecessary due to restricted mobility. Device checks at implant were all within acceptable ranges and a threshold of 0.4V at 0.5 ms was achieved with an average R wave of 15 mV. Radiographs confirmed correctly positioned right-ventricular lead and pulse generators and no complications were documented. At 1 day and 1 week post-implant follow-up checks the wound was healing well and no further problems were documented.
Implantation methods
It is widely accepted that the use of a pacemaker for such patients is a beneficial therapy that improves both quality and length of life (Epstein et al, 2008). Studies investigating the prognosis of patients with third-degree AV block and no pacemaker indicate 5-year survival rates of approximately 37–67% (Edhag and Swahn, 1976; Schmidt, 2003), although the rates are worse for patients aged over 80 years.
Guidance from the National Institute for Health and Care Excellence (NICE) advises that most cases of AV block warrant a dual-chamber pacemaker to improve exercise capacity and reduce pacemaker syndrome and atrial fibrillation (NICE, 2014). Pacemaker syndrome occurs when there is AV dyssynchrony, resulting in a fall in cardiac output and an increase in filling pressures, and is associated with reflex vasodilation (Lamas and Ellenbogen, 2004). However, there is little difference in mortality rates between single or dual-chamber devices and some studies have concluded that elderly patients with AV block receive little benefit from a dual-chamber over a single-chamber device (Gribbin et al, 2005; Toff et al, 2005). Arguably, dual-chamber devices have been indicated to improve quality of life in patients with AV block (Yee et al, 1984; Kristensson et al, 1985; Rediker et al, 1988; Menozzi et al, 1990; Oldroyd et al, 1991; Sulke et al, 1991; Linde-Edelstam et al, 1992; Lau et al, 1994; Lukl et al, 1994); however, in this case it was considered unlikely due to the patient's extremely low levels of movement and cognitive impairment. A single-chamber device was selected due to patient frailty and comorbidities in this instance, in accordance with the NICE (2014) guideline. Guidance from the manufacturer of the device used states that VVI mode can be conditionally indicated in patients with symptomatic bradycardia where prolongation of life is the primary objective (Biotronik Inc, 2011).
Creating as small a pocket size within the chest to house the device as possible and suturing it to the underlying fascia are highly recommended for minimising repeated twiddler's syndrome (Fahraeus and Höijer, 2003; Nicholson et al, 2003; Aliyev et al, 2009; Munawar et al, 2011); both methods were used for this patient's new implant. Some centres prefer to use active-fixation leads or Dacron patches (Furman, 1995) to stabilise the leads and generators by promoting tissue growth to anchor them in place. In this instance, the patient was unaware of previous damage caused, and an active-fixation lead was considered inappropriate, as in previous cases of twiddler's syndrome where such leads were employed they were not always successful and there was a risk of increased damage (Lal and Avery, 1990; Udink ten Cate et al, 2012). Dacron patches are not currently used at the centre where this case occurred, but it is hoped that the method of fixing the device in place achieves a similar result.
To minimise patient manipulation of the new device the unconventional decision to leave the old generator in situ, with settings designed to reduce the chances of complication (VVI mode 30 bpm, threshold 0.2V at 0.1 ms), was taken rather than extraction. No lead interaction could occur due to complete retraction, and no infection was present, diminishing the risks of abandoning the old device. If the device had been extracted the patient would have suffered a lengthier operation and another incision, invoking ethical issues regarding an extended procedure in a distressed patient, as well as increasing the potential for infection in a frail elderly woman. It would arguably be a simpler extraction compared with a lead still fixed within the cardiac tissue, thereby reducing some risks, but the potential for thrombus and venous damage meant that extraction was considered inappropriate.
It is uncommon for a generator to be abandoned because few scenarios call for such action, however, the rate of complications arising from abandoned leads has been shown to be 5.5%, relating to infections and vascular occlusions (Suga et al, 2000). Skin erosion has been reported as a complication in devices left in situ at a rate of about 5% (Böhm et al, 2001) and is arguably an amplified risk in this case; therefore increased follow-up surveillance was indicated.
Evidence from Glikson et al (2009) suggests that non-functional device abandonment shows no clear increase in risk of complications, however, follow-up was only over a short period of time. A review of extraction practices by Henrikson (2010) advised that a blanket approach was unlikely to be in patients' best interests and that a case-by-case evaluation of the risks of extraction compared with abandonment should be made. In this case the patient continued to manipulate her old device, therefore it was considered in her best interests to abandon it, but that if any issues arose extraction should be considered. It is hoped that if manipulation continues it will affect the old device only—because this is what the patient habitually rotates—rather than the new device, which would not be easy to manipulate due to the fixation methods used.
Consent in situations where patients have reduced capacity can be a difficult process to navigate. Where patients are deemed to lack capacity other people may make the decision for them, a process that can be legally agreed through a lasting power of attorney.
Follow-up
This case highlights the importance of regular comprehensive follow-up checks in detecting and managing postoperative complications. Physiologists check for device malfunctions and look for other problems (eg wound deterioration), and provide patients with information and advice (Jones et al, 2008). Early appointments are vital for tailoring any device to the patient and optimising functions to ensure longevity. At both 1-day and 1-week checks the pacemaker was working effectively, however, failure to attend subsequent appointments resulted in complications and presentation at A&E. Although research indicates that complications from reel syndrome are most likely to occur within the first month following implantation (Munawar et al, 2011) before tissues have secured the lead, it is unclear in this instance when device function was affected. However, cases of twiddler's syndrome have been reported as common at any time within the first year (Solti et al, 1989; Chauhan et al, 1994; Newland and Janz, 1994; Abrams and Peart, 1995; Higgins et al, 1998; Bracke et al, 2005). The 2012 FOLLOWPACE study (Udo et al, 2013) indicated that the majority of complications occur within the first 6 months and the rate of incidence declines steadily after this, highlighting the importance of initial appointments. The regularity of follow-up appointments is largely at the discretion of each centre but an early surveillance period, as recommended by Roberts (2005), would highlight device issues arising within the initial vulnerable 6 months.
Increasing use of remote device follow-up is improving early detection in this patient population, and may be useful in cases where vulnerable patients fail to attend scheduled appointments as a tool to indicate whether the device is working appropriately. Early detection of twiddler's syndrome has been documented through home monitoring (Sholton, 2004).
Patient involvement and understanding are integral to pacemaker management because they are likely to be the first to notice complications. However, in patients with cognitive impairment there may be a lack of understanding or ability to retain information, making it important for third parties, such as relatives or care home staff, to be aware of signs to watch and understand correct postoperative management. Despite this, signs of twiddler's syndrome, such as abnormal nerve stimulation resulting in twitching (Pereira et al, 1999; Nicholson et al, 2003) or a return of symptoms, may go undetected if carers associate these with patient comorbidities rather than recognising a device issue. This would be compounded if patients are unable to understand or communicate what they are experiencing, and may have been further exacerbated by the patient's frailty and multiple comorbidities. These comorbidities therefore amplified the possibility of postoperative complications, in addition to being at increased risk of developing twiddler's syndrome, which has a greater rate of incidence in patients who are female, elderly, overweight or have mental impairment (Fahraeus and Höijer, 2003; Aliyev et al, 2009), all of which applied in this case. Even with identified risk factors, recognising patients likely to be affected by twiddler's syndrome is not straightforward. Many pacemaker recipients have one or more of the risk factors, and therefore mental health problems are not a simple indicator of complications. Additionally dementia specifically is reportedly underdiagnosed within the primary care setting (Connolly et al, 2011) and therefore using this as an indicator of complications is inherently difficult. Those with unimpaired cognitive abilities can be informed of the dangers where twiddler's syndrome is suspected but those with severe mental health problems are at increased risk due to potential difficulties in relaying such hazards.
It is widely accepted that cognitive impairment, and dementia specifically, increases the risk of twiddler's syndrome (Newland and Janz, 1994; Fahraeus and Hoijer, 2003; Gupta and Lin, 2004; Castillo and Cavusoglu, 2006; Aliyev et al, 2009; Barold and Stroobandt, 2009), indicating that increased surveillance of such patients may be an appropriate approach. BJN