https://doi.org/10.1007/s00392-025-02625-4
1Universitätsklinikum Tübingen Innere Medizin III, Kardiologie und Angiologie Tübingen, Deutschland; 2Universitätsklinikum Tübingen Klinik für Thorax-, Herz- Gefäßchirurgie Tübingen, Deutschland; 3Universitätsklinikum Tübingen Innere Medizin III, Kardiologie und Kreislauferkrankungen Tübingen, Deutschland
Background: Brugada syndrome (BS) is associated with an increased risk of sudden cardiac death due to ventricular arrhythmias and ventricular fibrillation (VF). The extravascular implantable cardioverter-defibrillator (EV-ICD) is an alternative especially for young patients to prevent sudden cardiac death and provides in comparison to subcutaneous ICD systems pacing therapies (e.g. ATP). Defibrillation threshold testing (DFT) during implantation is recommended. Brugada patients are known to have higher rates of unsuccessful internal defibrillation. So far, no data exists on the safety and efficacy of the novel EV-ICD in this patient population.
Summary: We report a case of a 36-year-old asymptomatic man with BS who underwent implantation of an EV-ICD for primary prevention. The patient had a family history of sudden cardiac death in his newborn daughter and pathogenic variant in SCNA5 associated with BS. Ajmalin testing revealed a typical type 1 ECG. During the procedure of EV-ICD implantation induced VF could not be terminated by the device with up to 40 joules despite repositioning of the generator. Finally, external defibrillation with 360 joules terminated the episode. Three weeks after surgery we successfully repeated DFT testing proving shock efficacy with 20 joules.
Discussion: BS is associated with elevated DFT. In our case although initial DFT testing failed repeated testing after wound healing proved safe function of the EV-ICD. We have therefore shown that for BS patients EV-ICD can be a reliable treatment to prevent sudden cardiac death.