Background: The Brugada syndrome (BrS) is an inherited disorder associated with the risk of ventricular fibrillation and sudden cardiac death (SCD). The current main therapy is an implantable cardioverter-defibrillator (ICD). However, the risk stratification and management of patients remain challenging. Here, we present a case of BrS representative of the pitfalls that clinicians may encounter in the management of Brugada patients in routine clinical practice.
Case summary: A 39-year-old man with BrS and recurring syncope was implanted with a subcutaneous ICD (S-ICD) (EMBLEM MRI S-ICD, Boston Scientific). Syncope recurred some months later. Subcutaneous ICD interrogation showed no arrhythmic events, but SMART Pass (high-pass filter) deactivation was noted. A query was sent to Boston Scientific clinical service, unveiling an extremely long asystolic pause as syncope determinant. Subcutaneous ICD was explanted and replaced by conventional single chamber ICD in the pre-pectoral region.
Discussion: Brugada syndrome patients with high-risk features are candidates for ICD implantation to prevent SCD. Recent evidence highlighted that symptomatic patients carry a substantially higher risk compared with asymptomatic ones. Syncope may represent a pivotal symptom in BrS patients, but young patients with Type 1 Brugada pattern may experience syncope other than from tachyarrhythmias. Subcutaneous ICD is an advisable option in young ICD recipients to avoid lifetime complication related to standard transvenous systems. However, S-ICD lacks pacing capabilities and, therefore, is not indicated when an anti-bradycardia system is needed. The diagnostic workup of syncope in Brugada patients may be ineffective in elucidating the underlying aetiology whose understanding is essential to offer a personalized therapeutic approach.
Keywords: Asystole; Brugada syndrome; Case report; Subcutaneous implantable cardioverter-defibrillator; Syncope.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.