Twiddler's Syndrome

Overview

Twiddler's syndrome is a rare but serious complication that occurs in patients with implanted cardiac devices such as pacemakers or implantable cardioverter-defibrillators (ICDs). The condition is caused by the patient’s unintentional or habitual manipulation (twiddling or rotating) of the implanted device within its subcutaneous pocket. This leads to dislodgment or twisting of the device leads (wires), resulting in malfunction of the pacemaker or ICD. Twiddler’s syndrome can lead to failure of the device to deliver pacing or shocks, potentially resulting in life-threatening arrhythmias, syncope, or even cardiac arrest if not promptly recognized and managed.

Causes

Twiddler's syndrome is primarily caused by mechanical manipulation of the implanted device, either knowingly or unknowingly. Common contributing factors include:

• Patient behavior: Conscious or unconscious manipulation of the pacemaker/ICD generator, often out of habit, anxiety, or curiosity.

• Loose device pocket: A large or poorly secured subcutaneous pocket allows excessive movement of the device.

• Inadequate fixation: Improperly secured leads or device at the time of implantation increases the risk of lead dislodgment with movement.

• Older age or cognitive impairment: Elderly patients or those with dementia may be more likely to fiddle with the device unknowingly.

• Obesity: Excess soft tissue can make the device more mobile within the pocket.

Symptoms

The symptoms of Twiddler’s syndrome arise due to malfunction or dislodgment of the pacemaker or ICD leads. Clinical presentation varies depending on the function and indication of the device:

• Palpitations or dizziness: Due to ineffective pacing or failure of the device to regulate heart rhythm.

• Syncope or near-syncope: Caused by sudden loss of pacing or arrhythmia control.

• Inappropriate shocks (in ICD patients): Misinterpretation of signals due to lead displacement may lead to unnecessary defibrillator shocks.

• Muscle twitching: Stimulation of nearby muscles (e.g., chest wall, diaphragm, or brachial plexus) due to misplaced leads.

• Asymptomatic: In some cases, patients may remain asymptomatic until routine device checks reveal abnormalities.

Diagnosis

Timely diagnosis of Twiddler’s syndrome is crucial to prevent severe complications. The following steps are typically involved:

• Clinical history: Inquiry about symptoms, device awareness, and any habitual manipulation of the implant site.

• Physical examination: Palpation may reveal rotation of the device within the pocket or abnormal positioning.

• Device interrogation: Evaluation using a pacemaker or ICD programmer can reveal abnormal lead parameters, such as:

  • Increased pacing thresholds

  • Loss of capture

  • Lead impedance abnormalities

• Chest X-ray: A critical diagnostic tool showing twisted leads, lead retraction, or abnormal lead position in the heart or other areas.

• Electrocardiogram (ECG): May show loss of paced rhythm or inappropriate pacing behavior.

Treatment

The treatment of Twiddler’s syndrome involves correcting the lead displacement and preventing recurrence. Standard management includes:

• Surgical intervention:

  • Lead repositioning or replacement under fluoroscopic guidance.

  • Securing the device more firmly within a smaller or new subcutaneous pocket to prevent movement.

  • Use of non-absorbable sutures or anchoring sleeves to stabilize the leads and device.

• Patient education:

  • Informing patients about the importance of avoiding manipulation of the device area.

  • Providing written instructions and routine counseling, especially for high-risk individuals (elderly, cognitively impaired).

• Psychological evaluation: In cases involving compulsive behavior or psychiatric illness, referral to mental health professionals may be appropriate.

Prognosis

With prompt recognition and appropriate surgical correction, the prognosis for patients with Twiddler’s syndrome is generally good. Key prognostic points include:

• Favorable outcome: Most patients recover well after lead revision and stabilization of the device.

• Risk of recurrence: Recurrence can occur if patient behavior persists or if the device remains inadequately secured.

• Potential complications: Delayed diagnosis can lead to serious events such as cardiac arrhythmias, heart failure, or sudden cardiac death in high-risk patients.

Long-term follow-up and patient adherence to care instructions are essential to ensure continued device function and prevent recurrence of Twiddler's syndrome.