EP News: Case Reports

Congenital short QT syndrome is a cardiac channelopathy with mutations identified in the KCNH2, KCNJ2, and KCNQ1 genes. The syndrome is associated with atrial and ventricular arrhythmias and typically manifests early with sudden death or aborted cardiac arrest. During an electrophysiology study, induction of ventricular fibrillation (VF) can be seen in the majority of patients and they display unique susceptibility to mechanical stimuli. Farag and Atallah (DOI: https://doi.org/10.1016/j.hrcr.2018.11.015) shared a case of a 7-year-old boy with short QT syndrome and recurrent VF despite flecainide and amiodarone. An electrophysiology study was performed to identify and potentially ablate triggers of VF. Single ventricular extrastimuli from catheter manipulation recurrently provoked atrial fibrillation. An implantable cardioverter-defibrillator was recommended and implanted. Of interest, with any mechanical stimulation of the epicardium during lead implantation, the patient experienced recurrent VF. The surgeons sprayed 2% lidocaine over the surface of the heart and waited 2 minutes before resuming lead implantation. After treatment, the team was able to complete the operation without adverse events or further arrhythmias. The patient had no further events with device assessment over the ensuing 5 years. This case highlights the mechanical susceptibility in patients with short QT syndrome and provides a potential therapy when surgical manipulation is required in children who receive epicardial leads.