The role of adrenaline in comprehensive management of electrical storms in high-risk Brugada syndrome with rare SCN5A mutation: a case report from medical stabilization to implantable cardioverter-defibrillator implantation.

Background: Patients with Brugada syndrome (BrS) have a risk of sudden cardiac death, often linked to mutations in the SCN5A gene. Electrical storms (ESs) in this population are life-threatening and typically require urgent administration of isoproterenol; however, in resource-limited settings, alternative pharmacologic strategies may be required. The management of ES in BrS is complex and often relies on case reports due to the rarity of the condition.

Case summary: A 50-year-old male with no prior medical history presented with sudden cardiac arrest during a social event. Following successful resuscitation and multiple defibrillations for ventricular fibrillation, the patient was transferred to our centre with an ECG showing a spontaneous type 1 Brugada pattern. Despite therapy with multiple antiarrhythmic agents including amiodarone and lidocaine, the patient experienced recurrent episodes of ventricular fibrillation. In the absence of isoproterenol, low-dose adrenaline infusion was initiated, resulting in prompt termination of ES. A single-chamber implantable cardioverter-defibrillator was subsequently implanted in combination with quinidine and cilostazol for long-term suppression of arrhythmic events and prevention of recurrent ES. Genetic testing revealed two SCN5A mutations [c.4744C>T (p.Arg1582Cys) and c.3067C>T (p.Arg1023Cys)]; the first has not been previously reported in BrS.

Discussion: This case underscores the potential utility of adrenaline as an accessible and effective alternative in the acute management of ES in BrS patients, if isoproterenol is unavailable. It also highlights the importance of genetic evaluation in BrS for tailored therapeutic strategies.