Twiddler syndrome

Case contributed by Dr Ashesh Ishwarlal Ranchod


The patient presents with a 2-week history of the placement of a new dual chamber cardiac pacemaker and the onset of focal muscle twitching.

Patient Data

Age: 60 years
Gender: Female

The patient has a left subclavian venous access, dual chamber, and external cardiac pacemaker in situ. This is a dual lead device with the non-fragmented right atrial appendage lead correctly sited. There is extracardiac dislodgment and retraction of the non-fragmented right ventricular lead, with the tip lying within the left subclavian/axillary vein, mid-clavicular line. There is an enlarged cardiac silhouette consistent with a history of chronic hypertension. There is evidence of pulmonary edema and cardiac decompensation. The lung fields are hyperinflated but clear.

Case Discussion

The five common complications of permanent external cardiac resynchronization devices and pacemakers include hematoma, lead dislodgement, infection, lead perforation and dysrhythmias.

This is an example of lead dislodgement and extracardiac retraction of the right ventricular lead. Lead dislodgement most commonly occurs within the first three months of implantation and most commonly occurs in patients over 75 years of age. In this instance, there is a redundant lead wrapped around the pacemaker pulse generator device suggestive of Twiddler syndrome due to ongoing manipulation of the pulse generator in its subcutaneous pocket by the patient.

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