Where is the Lead? Inappropriate Implantable Cardioverter-Defibrillator Shock Caused by Extreme Twiddling

A 43-year-old man who had received mitral and aortic valve replacement surgery underwent the implantation of an implantable cardioverter defibrillator (ICD) for sustained ventricular tachycardia. The patient presented with a sudden jolting sensation in his left upper chest area one year after the device implantation. He had a history of vigorous upper body exercise during the several months of the follow-up period. Device interrogation revealed complete sensing and capture failure. The ventricular lead impedance was in the normal range, but the high voltage impedance had dropped to less than 10 Ω. Four inappropriate shocks for ventricular fibrillation had been delivered due to over-sensing of the atrial signal on the ventricular lead. Chest radiography showed ventricular lead displacement with extreme rotation and flipping-over of the generator. In the lead revision operation, the old ventricular lead was extracted and replaced, and the generator was fixed more deeply in the pocket with a non-absorbable ligature.

Twiddler’s syndrome in patient with single chamber pacemaker.

Twiddler’s syndrome is a well-known complication of pacemaker treatment. This syndrome is characterized by coiling of the pacemaker lead due to the rotation of pacemaker generator on its long axis. Lead damage could cause lead facture or insulation leakage. The syndrome is also responsible for lead dislodgment, diaphragmatic stimulation, twitching upper arm due to plexus brachial nerve stimulation and loss of capture. Understanding risk factors and preventive measurement is very important. In this case report we present an 84 year-old patient who managed to rotate his single chamber pacemaker generator unintentionally following implantation in which the syndrome occurred within 2 months causing insulation leakage and battery depletion. For the best of our knowledge, this is the first report of twiddler’s syndrome in Indonesia.

Síndrome de Twiddler: una causa infrecuente de disfunción de marcapaso definitivo. Caso clínico / Twiddler syndrome: Report of one case

We report a 26year old patient who had a single chamber pacemaker implantation one year before. During a routine pre-operative evaluation, pacemaker dysfunction was demonstrated due to sensing and pacing failure, associated to left pectoral muscle rhythmic contraction. Chest X-ray confirmed Twiddler syndrome, in which twisting or rotation of the device inside the pocket results in lead dislodgement and device malfunction.

A case of Defibrillator Twiddler's syndrome / 대한내과학회지

Here we describe a case of Twiddler's syndrome in a patient with an implanted cardioverter-defibrillator who tinkered with the device, shifting it to a vertical position and causing her skin to protrude in various locations. Fluoroscopy was used to determine what direction the device was rotating in during specific body postures and physical activities. Inappropriate shocks were delivered whenever the patient pushed the device down toward her sternum. The syndrome did not recur following training of the patient in a lead-revision procedure that involved shifting the device down vertically toward her lateral side with her hands.

A Case of Recurrent Pacemaker Twiddler's Syndrome

Pacemaker twiddler's syndrome is reported as a very rare complication of permanent pacemaker implantation. There was a recent report suggesting that the incidence of pacemaker twiddler's syndrome increase recently presumably as a result of the implantation of thinner and smaller pacemaker system than before. We experienced a case of pacemaker twiddler's syndrome complicated 3 times with the conventional method of implantation or replacement during 14 months after the first implantation(Optims MP 158C and Pacing lead 400, Telectronic)on June 13th 1987. This case was an 18 year-old high school girl who had suffered frequent syncope for 2 years and extertionl dyspnea for 5 years due to congenital complete heart block, of which block site was proved to be AV nodal by His bundle electrogram. Pacemaker twiddler's syndrome developed 3 times;firstly 6 weeks after the first implantation in the right subclavicular fossa, secondly 10 weeks after the replacement of the twisted pacing lead, thirdly 10 months after the change of implantation site to the left subcalvicular fossa with the replacement of the twisted and fractured lead. Finally, the pacemaker generator was anchored to the clavicular periostium and pectoralis fascia at several points by using Dacron pouch.