Case Report: Twiddler’s Syndrome

Eleonore Werner, MD, Matthew Kirk, BSRS, RT(R), RCIS, David Rancourt, DO, MPH, Clayton Shaker, MD, Brett Hiendlmayr, MD, Suleiman Lapalme, MD Department of Cardiology Maine Medical Center Portland, Maine
Eleonore Werner, MD, Matthew Kirk, BSRS, RT(R), RCIS, David Rancourt, DO, MPH, Clayton Shaker, MD, Brett Hiendlmayr, MD, Suleiman Lapalme, MD Department of Cardiology Maine Medical Center Portland, Maine

Abstract

Twiddler’s Syndrome, first described in 1968, refers to permanent malfunction of a pacemaker secondary to manipulation of the pulse generator within its pocket.1 This leads to ineffective ventricular pacing due to displacement of the leads. This syndrome most commonly occurs in elderly, obese, and psychiatrically ill patients2 within the first six months after ICD implantation; however, it can occur at any time, and can lead to eventual cardiovascular compromise if not treated promptly.

Here we report a case of a female patient who presented to the hospital after a wireless monitor triggered an alert, revealing evidence of a lead fracture in her ICD. She presented to the hospital in atrial fibrillation (AF), and was admitted for telemetry monitoring and right ventricle lead revision. The patient underwent successful lead revision and was discharged with close follow-up monitoring.

Case Description

The patient is a 76-year-old woman with a past medical history significant for an apical variant of hypertrophic cardiomyopathy with chronic persistent AF and ventricular tachycardia (VT) status post implantable cardioverter defibrillator (ICD) implantation in 2000, with subsequent generator change in 2008. The patient presented to Maine Medical Center in December 2009 after her wireless device monitor triggered an alert, revealing evidence of a lead fracture. The device showed an abrupt increase in impedance in the superior vena cava (greater than 200 ohms) as well as increased impedance of the right ventricular coil. There was no evidence of high frequency noise on the lead to suggest paced sense fracture. Because of her reported history of appropriate VT/VF in the past due to shock coil lead failure, she was admitted for lead revision.

The patient’s past medical history was significant for chronic kidney disease, hypertension, dyslipidemia, restrictive lung disease, and diastolic heart failure. The patient reports a five-pound weight loss in the last two weeks, as well as increased shortness of breath. She also reports being bothered by the location of her implantable cardioverter-defibrillator (ICD). All other systems were negative.

Upon physical exam, her heart rate was 81 bpm, respiratory rate 16, blood pressure 127/71, SpO2 93% on room air. She had mildly elevated jugular venous pressure with no thyromegaly. Her cardiovascular review showed irregular rhythm and rate with a systolic ejection murmur that increases with Valsalva. ECG showed atrial fibrillation with controlled ventricular response, voltage criteria for LVH was present. 

The patient was admitted for telemetry monitoring and right ventricle lead revision. The patient remained in AF throughout the hospital stay; she had one episode of non-sustained VT of five beats, but remained asymptomatic. On day 3, the patient underwent successful right ventricle lead revision. During the procedure, visual inspection revealed that the device moved quite easily in its pocket and there was twisting of the superior vena cava lead consistent with Twiddler’s Syndrome. Once new leads were placed, the device was secured in the pocket with sutures. The patient tolerated the procedure without any complications and was discharged on day 4 with close follow-up.

Discussion

A majority of patients with Twiddler’s Syndrome do not admit to conscious manipulation of the ICD; however, many complain of a local discomfort or awareness that the generator is rotating in the subcutaneous pocket.3 It is hypothesized that it is the location of the generator that can induce subconscious manipulation. This rare syndrome, with a reported frequency of 0.07-7%, occurs most commonly when the generator is implanted in lax subcutaneous tissue (i.e., the abdomen of an obese patient). Careful suturing of the device to underlying fascia and matching the pocket size to the device size can help to decrease the risk of conscious or subconscious patient manipulation.

This case illustrates the presentation and management of Twiddler’s Syndrome. This unfortunate psychiatric predilection to twisting the ICD in its pocket can lead to rapid cardiovascular compromise and failure of the device to sense and treat an arrhythmia. Other adverse outcomes of lead displacement include possible stimulation of the ipsilateral phrenic nerve and brachial plexus causing diaphragmatic contractions and rhythmic arm twitching.2 Therefore, it is important to monitor all patients with implanted ICDs for the development of this syndrome. 

Disclosures: The authors have no conflicts of interest to report. 

References

  1. Bayliss C, Beanlands D, Baird R. The pacemaker-twiddler’s syndrome: a new complication of implantable transvenous pacemakers. Can Med Assoc J. 1968;99(8):371-373.
  2. Gkinos C, Manouras A, Lagoudianakis EE, et al. Twiddler’s syndrome. Hellenic J Cardiol. 2007;48(5):300-301.
  3. Navone A, Picone A, Boahene K. Defibrillator twiddler’s syndrome: a rare cause of implantable cardioverter-defibrillator failure. Heart. 1996;76:455-456.
  4. Mandal S, Pande A, Kahali D. A Rare Case of Very Early Pacemaker Twiddler’s Syndrome. Heart Views. 2012;13(3):114-115.