Journal of Rare Cardiovascular Diseases

Brugada syndrome is an autosomal dominant genetic disease with variable expression characterized by abnormal electrocardiographic findings – right bundle branch block and ST-segment elevation in the anterior precordial leads, without evidence of structural heart disease. Individuals affected by the disease have an increased risk of ventricular tachyarrhythmias and sudden cardiac death (SCD). We present a case of a 33-year old man who was admitted to cardiology department after sudden cardiac arrest due to ventricular fibrillation. Evaluation for a structural heart disease was negative and routine tests did not reveal the cause of cardiac arrest. Retrospective evaluation of the patient electrocardiograms showed changes consistent with Brugada type 2 pattern that were variable over time. Performed pharmacological challenge with sodium channel blocker – ajmaline – provided an ambiguous result. Nevertheless, the patient was qualified for an implantable cardioverter defibrillator implantation as a secondary SCD prophylaxis. He underwent the procedure without complications and was discharged home in good general condition without symptoms.

rare disease; electrocardiography; ajmaline challenge; ventricular arrhythmias; implantable cardioverter‑defibrillator

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