Twiddler's Syndrome: Case Report and Literature Review.

Twiddler's syndrome is a rare complication after pacemaker implantation usually caused by patient manipulation with generator. We describe a case of 70-year-old female patient with pacemaker who was admitted to the neurological clinic with syncope and suspicion for neurological disease. After neurological diagnostic tests that were negative and consultation with a cardiologist, Twiddler's syndrome was diagnosed.

INTRODUCTION

Twiddler's syndrome was described 50 years ago by Bayliss et al.[1] It is a very serious state caused by patient's mechanical manipulation of the pacemaker generator and can be manifested without or with symptoms such as syncope, vertiginous disorders, fatigue, abdominal pulsation due to nerve stimulation, rhythmic arm movements due to brachial plexus stimulation, chest pain (sometimes described as an intermittent “firing/shock in chest”). It can also lead to death of the patient.[234]

We present a patient with Twiddler's syndrome who was admitted to the neurological clinic because of suspicion that the symptoms have neurological etiology.

CASE PRESENTATION

A 70-year-old female patient was admitted to the neurological clinic with recurrent presyncope and syncope, vertiginous disorders, headache, nausea, and vomiting. These symptoms started 2 months after implantation of pacemaker according to her anamnesis (in medicine anamnesis is a patient's account of their medical history). A single chamber ventricular pacemaker with satisfactory lead parameters for sensing and pacing was implanted (sensing R wave 12 mV, capture threshold 0.250 at 0.40 ms, impedance 1500 Ω).

Her medical history was negative for the coronary heart disease, neurological diseases. Physical examination was unremarkable, with a heart rate of 60 beats/min, and blood pressure 150/80 mmHg. No auscultation abnormality.

The first electrocardiography (ECG) showed regular ventricular paced rhythm. Neurological examination was negative but with positive Romberg's test. Due to suspicion of the stroke, the computed tomography of the head was done. Her CT scan was without any visible pathomorphological changes. Furthermore, electroencephalogram was done, but it was normal.

After consultation with the cardiologist about further therapy, the pacemaker interrogation was suggested and done. It showed increase and instability in the capture threshold of the ventricular lead which was started approximately 2 months after pacemaker implantation [Figure 1].

Figure 1

Pacemaker interrogation

Another ECG strip was done and intermittent failure of appropriate ventricular capture was recorded [Figure 2]. Furthermore, the chest X-ray was performed and it showed the displacement of the ventricular lead in the right ventricle with twisted lead near generator [Figure 3]. The diagnosis of pacemaker Twiddler's syndrome was made. She was taken immediately to the catheterization laboratory, and the new screw-in ventricular lead was inserted [Figure 4]. The generator and new lead were fixed with nonabsorbable suture below the pectoral muscle.

Figure 2

Electrocardiography strip with one failure of appropriate ventricular capture

Figure 3

Chest X-ray with displacement of ventricular lead in the right ventricle and twisted lead near generator

Figure 4

Intraoperative image showing twisting of the lead near pacemaker generator

After the intervention, the patient told that she did not manipulate with pacemaker, but her son told that she had mental problems in that period and that she was very upset. Two days later, after consultation with psychiatrist, she was released from the hospital without any complications.

DISCUSSION

Twiddler's syndrome is a very rare complication after the pacemaker implantation with frequency around 0.07%–7%,[5] which is mostly caused by patient's manipulation with pacemaker generator. Consequence of manipulation with the generator is dislocation and/or fracture of the leads.[2345678] This syndrome occurs usually in the 1st year after implantation as in our case, but it can occur later.[10] It is not only associated with cardiac devices but it can also happen with central venous catheter.[11] Twiddler's syndrome is especially dangerous in patients with defibrillator because of possible false treatment of malignant ventricular arrhythmias and inappropriate shocks due to the loss of adequate sensing and capture.[78]

Fast diagnosis and treatment is of the vital importance. The ECG, chest X-ray, and pacemaker interrogation can provide us with the necessary information, and they should be standard diagnostic tools for the diagnosis of Twiddler's syndrome. Chest X-ray gives a clear image of the lead coiling and device rotation.[41112] Remote monitoring and follow-up with automatic alerts for arrhythmias, changes in pacemaker basic parameters can also provide necessary information about the malfunction of the cardiac device.[13]

According to our opinion, there are two very important facts to consider in how to prevent Twiddler's syndrome. The first is that the patients who are candidate for pacemaker are not all the same and that Cardiologist should pay attention to high risk patients for development of Twiddler's syndrome: Female, elderly and obese patients, pediatric patients, patients with mental disorders, as well as the patients who are in risk for psychoorganic syndrome.

The second fact which is also important is the education of the patient and family about life with pacemaker and similar devices. These devices are created to help and also to improve quality of life but with the wrong manipulation, they can be very dangerous for the patient. As we showed in our case twiddling with pacemaker generator caused syncope and other symptoms which can also be neurological etiology, but it could also lead to fatal cardiac arrhythmia and device failure. It is necessary that the medical staff provide all the information about the pacemaker and how to live with pacemaker.

Appropriate implantation technique is one of the solutions for Twiddler's syndrome. Active fixation of leads can be used; making a small pocket and suturing device on the pectoral muscle with nonabsorbable sutures and implantation of the device under the pectoral muscle.[356911] Furthermore, some authors also suggest the use of Dacron patch which promotes tissue growth around the device.[6]

CONCLUSION

We have presented a case of the Twiddler's syndrome that is unique because it was first misdiagnosed as neurological disease. Most of the patients with this syndrome are diagnosed within the 1st month of implantation during routine pacemaker control. Timely detection of the patients who are at risk for development of the Twiddler's syndrome and their education in the prevention remains of crucial importance.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.