Abstract 13000: Twiddler’s Syndrome - A Rare Cause of Pacemaker Malfunction

Abstract

Description of Case: A 76-year-old female with a past medical history of chronic atrial fibrillation and symptomatic tachycardia and bradycardia status-post dual-chamber pacemaker with right atrial and ventricular leads who presented with dyspnea on exertion. TTE showed normal biventricular function and no significant valvular abnormalities. Upon further evaluation of her pacemaker, she was found to have a very high ventricular capture threshold suggesting RV lead failure. The patient underwent RV lead replacement. The inspection of the pacemaker pocket during the procedure showed manual dislodgement of both tie-down sleeves and the pacemaker leads required untangling. A new RV lead was subsequently placed. Upon further imaging review, displacement of the previous RV lead was evident from initial placement. In addition, the patient had previously had a history of interval diaphragmatic stimulation as well. Overall, the presentation was felt to be consistent with Twiddler’s Syndrome. Discussion: Twiddler’s or Reel Syndrome occurs when conscious or subconscious self-manipulation and spinning of the pulse generator result in lead dislodgement and loss of pacing and pacemaker malfunction. The reeling of the leads around the generator can also cause stimulation of the brachial plexus or phrenic nerve stimulation and subsequent rhythmic arm twitching or diaphragmatic pacing, respectively. It has previously been reported in cases of single- and dual-chamber ICDs, deep brain stimulators, and spinal nerve stimulators with varying presentation. The diagnosis requires clinical suspicion based on history, physical exam, imaging, or surgical inspection suggesting the displacement of leads with evidence of coiling of the wires around the pacemaker. Treatment often requires revision and focus should be maintained on patient education for both primary and secondary prevention of Twiddler’s Syndrome.