Advances and Challenges in the Management of Brugada Syndrome: A Comprehensive Review

Brugada syndrome (BrS) is an inherited arrhythmogenic disorder marked by distinctive ST-segment elevations on electrocardiograms (ECG) and an increased risk of sudden cardiac death. Characterized by mutations primarily in the SCN5A gene, BrS disrupts cardiac ion channel function, leading to abnormal...

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Published in:Curēus (Palo Alto, CA) CA), 2024-06, Vol.16 (6), p.e61837
Main Authors: McBenedict Billy, Berley, Alphonse, Devan, Jeshua N, Singh, Gurinder, Goh Kang Suen, Yau Ryan Chun Chien, Elamin, Sara, Vaishnavi, Jamched, Abraham, Aaron A, Valentim Gabriella, Lima Pessôa Bruno, Hauwanga, Wilhelmina N
Format: Article
Language:English
Subjects:
Ablation
Asymptomatic
Cardiac arrest
Cardiac arrhythmia
Cardiology
Catheters
Family/General Practice
Genes
Genetic testing
Internal Medicine
Mutation
Sodium
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Summary:Brugada syndrome (BrS) is an inherited arrhythmogenic disorder marked by distinctive ST-segment elevations on electrocardiograms (ECG) and an increased risk of sudden cardiac death. Characterized by mutations primarily in the SCN5A gene, BrS disrupts cardiac ion channel function, leading to abnormal electrical activity and arrhythmias. Although BrS primarily affects young, healthy males, it poses significant diagnostic challenges due to its often concealed or intermittent ECG manifestations and clinical presentation that can mimic other cardiac disorders. Current management strategies focus on symptom control and prevention of sudden death, with implantable cardioverter-defibrillators (ICD) serving as the primary intervention for high-risk patients. However, the complications associated with ICDs and the lack of effective pharmacological options necessitate a cautious and personalized approach. Recent advancements in catheter ablation have shown promise, particularly for managing ventricular fibrillation (VF) storms and reducing ICD shocks. Additionally, pharmacological treatments such as quinidine have been effective in specific cases, though their use is limited by availability and side effects. This review highlights significant gaps in the BrS literature, particularly in terms of long-term management and novel therapeutic approaches. The importance of genetic screening and tailored treatment strategies to better identify and manage at-risk individuals is emphasized. The review aims to enhance the understanding of BrS and improve patient outcomes, advocating for a multidisciplinary approach to this complex syndrome.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.61837