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Fever, Syncope, and the Brugada Dilemma: Navigating the Complexities of ICD Decision-Making in an Atypical
Lorna Devkota1, Michael Hoffer-Hawlik1, Felix Yang2
1Department of Medicine, New York University Grossman School of Medicine, New York, New York, USA.
Insights
Brugada syndrome (BrS), a rare inherited heart condition, can be unmasked by fever, leading to arrhythmias. This case highlights challenges in diagnosing and managing BrS, even with a negative drug challenge.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Brugada syndrome (BrS) is a rare inherited cardiac channelopathy linked to sudden cardiac death (SCD).
- Diagnosis relies on a Type 1 electrocardiogram (EKG) pattern, characterized by coved ST-segment elevation.
- Fever is a known trigger that can unmask BrS by exacerbating sodium channel dysfunction.
Abstract:
Brugada syndrome (BrS) is a rare inherited cardiac channelopathy associated with ventricular arrhythmias and sudden cardiac death (SCD), often in individuals with structurally normal hearts. It is diagnosed by a Type 1 electrocardiographic (EKG) pattern-coved ST-segment elevation in the right precordial leads. Fever is a known trigger that can unmask Brugada patterns by worsening sodium channel dysfunction. We present the case of a 20-year-old male with a fever and an episode of syncope prior to admission who had an EKG showing a Type 1 Brugada pattern. Procainamide challenge was negative, but an atypical right bundle branch block suggested sodium channel dysfunction. Despite the absence of structural heart disease, an implantable cardioverter defibrillator (ICD) was placed for primary prevention of SCD. This case underscores the diagnostic and management challenges in intermediate-risk BrS patients.
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