Lead system causing twiddler's syndrome in patients with an implantable cardioverter-defibrillator

Abstract

Three male patients with an implantable cardioverterdefibrillator (ICD) were referred for lead extraction because of twiddler’s syndrome associated with lead malfunction detected during routine follow-up (Table 1). The ICDs were implanted in the left subpectoral region in a submuscular pocket. On chest radiograph, the position of the generator was the same as at implant in all 3 patients. All patients were satisfied with the device and denied manipulation of the generator or discomfort at the implant site. All had a history of recent strenuous physical exercise. None of the patients was obese or reported important weight loss since the time of implant. We could not rotate the generator within the generator pocket in any of the patients, even during surgery, because the pocket fitted snugly in all. The leads were twisted tightly outside the generator pocket, and atrial and defibrillation leads were twirled around each other in the patients with a dual-chamber ICD (Figure 1). The torque of the leads had deformed the insulation, and in 1 patient there were insulation defects at the site of the connector and the yoke. The leads were extracted in all 3 patients, in 2 with a laser sheath. New leads were inserted in 2 patients through an exchange procedure and by renewed catheterization of the subclavian vein in the first patient. Care was taken not to leave any redundant lead outside the generator pocket, which was closed separately. The original ICD was reimplanted in all patients. One patient had recurrence of the syndrome. Seven months after the revision, the chest radiograph showed an unchanged position of the leads and generator. He then took up swimming but presented 3 months later with recurrence of twiddler’s syndrome. The other patients showed no recurrence during a follow-up of 10 and 16 months. Discussion Twiddler’s syndrome of ICDs is typically attributed to either conscious or unconscious rotation of the generator. The patients are often obese women with large defibrillator pockets, sometimes attributed to weight loss facilitating manipulation and rotation of