Twiddler’s syndrome with a subcutaneous implantable cardioverter-defibrillator presenting with an inappropriate shock: a case report

BACKGROUND: Subcutaneous implantable cardioverter-defibrillators (S-ICDs) are increasingly used in patients at risk of fatal cardiac arrhythmias. Twiddler’s syndrome is a condition in which a device is manipulated by the patient after implantation leading to lead twisting and retraction. Device manipulation has been reported multiple times in transvenous pacing systems and occasionally leads to inappropriate discharges from implanted defibrillators. However, little has been reported about device manipulation in S-ICD devices. CASE SUMMARY: We present the case of a 16-year-old who underwent insertion of an S-ICD for idiopathic dilated cardiomyopathy. He represented for a pacing check following a discharge from the device. This showed a significant change in the sensed vectors. Chest radiographs confirmed lead retraction and suggested device manipulation. The device was turned off to prevent further inappropriate shocks. The patient underwent successful reimplantation of a S-ICD device. DISCUSSION: This case highlights that twiddler’s syndrome can occur in those with an S-ICD and lead to an inappropriate device discharge. The patient in this case had a number of risk factors that have been previously associated with twiddler’s syndrome.