Twiddlers syndrome presenting as life threatening electrical storm

Area of Science:

• Cardiology
• Medical Device Technology
• Electrophysiology

Background:

• Implantable cardioverter-defibrillators (ICDs) are crucial for preventing sudden cardiac death in patients with severe cardiomyopathy.
• Lead integrity and proper positioning are essential for accurate signal sensing and effective defibrillation therapy.

Observation:

• A 75-year-old male with non-ischaemic cardiomyopathy developed inappropriate ICD shocks 13 months post-implantation.
• Over-sensing of the right ventricular lead and double counting of right atrial signals were identified as the cause.
• Chest X-ray revealed significant lead displacement: the right ventricular lead migrated to the right atrium with coiling in the pocket, and the shocking coil was near the tricuspid annulus.

Findings:

• The patient's symptoms were attributed to Twiddler's syndrome, a rare complication involving ICD lead dislodgement.
• Diagnosis was confirmed via chest X-ray, demonstrating the characteristic lead migration pattern.
• Successful management involved removal and replacement of the dislodged ICD lead.

Implications:

• Twiddler's syndrome can lead to inappropriate shocks and device malfunction, posing a significant risk to patient safety.
• This case highlights the importance of vigilant post-implantation monitoring and awareness of lead complication syndromes.
• Prompt recognition and intervention are critical for mitigating the potentially lethal consequences of ICD lead displacement.