Syncope and "Normal" ECG: Is Brugada Syndrome the Culprit?
Pamela Ramírez-Rangel1, Luis Emilio Bautista-Torres1, Miguel Angel Lopez-Lizarraga1
1Clinical Cardiology, National Institute of Cardiology Ignacio Chávez, Mexico City, MEX.
Insights
Brugada syndrome (BrS) is an inherited heart condition linked to sudden cardiac death. This case highlights a young man whose initial normal ECG evolved to a diagnostic Type 1 Brugada pattern, emphasizing the need to rule out phenocopies.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Brugada syndrome (BrS) is an inherited cardiac arrhythmia associated with sudden cardiac death (SCD) in structurally normal hearts, often linked to SCN5A gene variants.
- The diagnostic hallmark of BrS is the Type 1 electrocardiogram (ECG) pattern, characterized by specific J-point elevation and T-wave morphology in anterior leads.
Observation:
- The case involves a young male presenting with syncope and an initial non-diagnostic ECG.
- Subsequent ECG monitoring revealed the development of a Type 1 Brugada pattern.
- The patient also experienced an episode of non-sustained ventricular tachycardia.
Findings:
- The study underscores the dynamic nature of ECG manifestations in Brugada syndrome.
- It emphasizes the critical importance of differentiating true BrS from Brugada phenocopies, which mimic the Type 1 ECG pattern due to various conditions.
- The case illustrates the potential for syncope and ventricular arrhythmias in patients with evolving BrS.
Implications:
- Accurate identification of the Type 1 Brugada ECG pattern is crucial for timely diagnosis and management.
- Ruling out Brugada phenocopies is essential to avoid misdiagnosis and ensure appropriate patient care.
- This case highlights the need for vigilant ECG monitoring in patients with unexplained syncope, particularly when initial findings are equivocal.
Abstract:
Brugada syndrome (BrS) is an inherited disorder associated with a risk of ventricular fibrillation (VF) and sudden cardiac death (SCD) in a structurally normal heart mainly in young males, related to pathogenic variants in the SCN5A gene. There are three electrocardiographic patterns in BrS that must be identified, being diagnostic only type 1 pattern (coved J-point elevation >2 mm with a negative T-wave in V1 to V3). However, other circumstances can lead to a type 1 Brugada-like ECG, such as atypical right bundle branch block, pectus excavatum, arrhythmogenic right ventricular cardiomyopathy, acute coronary syndromes (occlusion of the left anterior descending artery or the conus branch of the right coronary artery), hypokalemia/hyperkalemia and cocaine poisoning, when present, these conditions are known as Brugada phenocopies, and it is imperative to rule out these conditions. We present the case of a young man with syncope with an initial suspected "normal" electrocardiogram with posterior development of type 1 Brugada pattern and an episode of non-sustained ventricular tachycardia.
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