Twiddler’s Syndrome Combined with Subclavian Crush Syndrome: A Case of ICD Lead Failure and Potential Challenging Lead Extraction

A 68-year-old woman with a body mass index of 37 kg/m² and a previous history of hypertension was referred to our department for transvenous lead extraction because of implantable cardioverter defibrillator (ICD) lead (Medtronic, active fixation) failure. In September 2017, she underwent single-chamber ICD implantation through the left subclavian vein for secondary prevention. In March 2018, the device emitted an audible alarm. Device control revealed significant reduction of lead impedance (41 Ohms) and complete deficit of pacing. Fluoroscopy revealed severe wrapping of the lead within the generator pocket and into the right atrium (Figure 1A; black arrow). Moreover, entrapment of the lead between the left clavicle and the first rib, suggesting a possible loss of the lead insulation, was observed, indicating a subclavian crush syndrome (Figure 1A; white arrow). She was taken to the operating room. Pocket revision revealed tangling leads near the generator (Figure 1B; black arrow) with insulation defects on the lead. It was immediately clear that it was impossible to retract the active fixation screw to advance the stylet to the lead tip, because of the lead fracture under the left clavicle. After prepping the lead with a Bulldog lead extender (Cook Medical), it was finally extracted after several attempts and adjustments using manual traction; there were no complications. A new, single-chamber ICD was then implanted from the left axillary vein. A Twiddler’s syndrome combined with subclavian crush syndrome was hypothesized. Our report demonstrates how this association may represent a challenging cause of lead failure and a potential cause of challenging transvenous lead extraction. Thus, this procedure should be performed in centers with experience with the appropriate tools. 

Reprinted with permission from J INVASIVE CARDIOL. 2019;31(11):E340.

Disclosures: The authors report no conflicts of interest regarding the content herein.