Twiddler’s syndrome after implantation of baroreflex activation therapy: a case report

Background Twiddler’s syndrome is a rare complication after implantation of cardiac pacemakers or cardioverter-defibrillators that usually occurs within the first year after the procedure. However, it has not yet been described following implantation of baroreflex activation therapy (BAT). Case summary A 61-year-old female patient was referred to the cardiology outpatient clinic due to uncontrolled arterial hypertension despite maximal doses of several established drugs. Therefore, right-sided BAT implantation was successfully performed in February 2017 with good clinical response. Because of sustained neck pain at the site of stimulator, surgical revision was performed in November 2019 including a switch of the lead to the contralateral position. Approximately 1 month later, Twiddler’s syndrome was identified on the basis of recurrent pain at the generator site necessitating pocket-revision, however, the lead was only untwisted but not replaced. A few weeks afterwards, unfortunately, lead revision was indispensable due to lead fracture. Discussion This case presents the uncommon phenomenon of Twiddler’s syndrome after BAT implantation. In addition, the commonly twisted lead should always be replaced as well during surgical pocket-revision in order to ensure proper long-term function.

Abstract 15480: Twiddler’s Syndrome in a Subcutaneous Implantable Cardioverter-Defibrillator

Introduction: Twiddler’s syndrome refers to deliberate or unintentional twisting of pulse generator in device pocket resulting in lead migration, which can present as malfunction of pacemaker or implantable cardioverter-defibrillator (ICD). In the past 50 years, twiddler’s syndrome with transvenous ICD and pacemaker has been well-described and thoroughly studied. However, there are very little data on twiddler’s syndrome with subcutaneous ICD (S-ICD). Case presentation: The patient is a 50-year-old male with a past medical history of hypertrophic cardiomyopathy, cardiac MRI showing left ventricular hypertrophy with a wall thickness of 24 mm, non-sustained ventricular tachycardia and evidence of late gadolinium enhancement scar, who underwent implantation of S-ICD for primary prevention. First follow-up 2 weeks post-implant showed appropriate device function. Two weeks later, remote monitor documented atrial fibrillation and ICD shocks. Surface ECG showed reduced R wave and delivered shock impedance was 7 ohms. Patient reported no symptoms and was clapping hands to music when it occurred. Chest X-Ray revealed S-ICD lead dislodged and retracted with coiling around the generator in the left lateral chest wall (Figure 1). Subsequently patient underwent lead revision and device replacement. To reduce the risk of future dislodgement, during re-implantation, the tip of the ICD lead was secured with 0-Silk. Conclusion: Twiddler’s syndrome is a rare complication after ICD implantation, not only with transvenous-ICDs but also involving S-ICDs. Risk factors include advanced and younger ages, redundant skin, obesity, oversized pocket, and psychiatric disorder. It can be a potentially dangerous and even fatal due to undetected or unsuccessful therapy for ventricular tachyarrythmias. Therefore, it is of paramount importance to diagnose and intervene early to avoid morbidity and mortality in these patients.