1Universitätsklinikum Mannheim I. Medizinische Klinik Mannheim, Deutschland; 2Universitätsklinikum Mannheim Med. Klinik IV Mannheim, Deutschland
Background and objectives:
Brugada syndrome (BrS) is a rare channelopathy, associated with an increased risk of ventricular fibrillation and sudden cardiac death (SCD). Syncope is a common symptom, but in up to 25% of cases, ventricular tachyarrhythmias (VA) are the first manifestation of BrS. The implantation of a cardioverter-defibrillator (ICD) may be considered for primary prevention in selected patients. However, a general recommendation only applies to secondary prevention after surviving SCD or documented VA.
Methods:
317 patients (64 % male) were initially diagnosed with Brugada syndrome (BrS) at our centre between 1998 and 2023. 295 patients were regularly followed at our centre. Of those, 121 patients (75% male) received an ICD. Outcome-relevant data such as appropriate and inappropriate ICD shocks, ICD-related complications (lead defects, infection, etc.) were collected.
Results:
Of 121 patients, 24 (20 %) received the ICD in terms of secondary prevention. Primary prevention patients were implanted due to a clinically increased probability of future arrhythmic events (spontaneous type 1 ECG, syncope, etc.). In the latter, patients were significantly older and more frequently family members of BrS patients. Both groups had equally often spontaneous type 1 ECG.
Follow-up data with a median of 116 months (IQR=143) was available.
45 ICD patients had a total of 114 shocks. Of these, 32 (28%) were appropriate (documented ventricular fibrillation) and 82 (72%) were inappropriate. 24 (29%) of all inappropriate shocks were caused by lead defects, 16 (20%) by atrial tachycardia or atrial fibrillation (AT/AF), 17 (21%) by mis-sensing (e.g., TWOS) and 24 (29%) by other or unknown causes.
The annual rate for complications (inappropriate shocks, lead failure, infection) was 14% for all ICD patients. Initially asymptomatic patients had the highest risk of complications with an annual rate of 19%. The annual rate for appropriate shocks was 2.6 % and was highest in patients with initially documented VA at 5.7 %.
Conclusion:
The rate of adverse ICD-related complications in BrS is high. Specifically asymptomatic patients had an increased risk of lead-related complications and inappropriate ICD shocks, while the rate of appropriate shocks was highest in the group of patients with previously documented VA. Primary preventive ICD implantation must be weighed against potential device-related complications despite progress in device programming.