Twiddler’s syndrome presenting as life threatening electrical storm

A 75-year-old man underwent implantation of a single chamber implantable cardioverter defibrillator (ICD) for primary prevention of his underlying severe non-ischaemic cardiomyopathy. Thirteen months later, he presented to the emergency room(ER) with inappropriate ICD shocks as a result of over sensing of the right ventricular lead and double counting of the right atrial signals. The chest X-ray (CXR) revealed a right ventricular ICD lead displaced into the right atrium with coiling in the pocket. The right ventricular shocking coil was noted at the tricuspid annulus. The lead was removed from the pocket and was replaced with a new lead. This case represents the classical Twiddler’s syndrome in an ICD with potential lethal consequences.