Key Points
- Confirm diagnosis early with highest‑yield tests (and do not let testing delay time‑critical therapy).
- Use explicit hemodynamic, respiratory, and neurologic targets to guide escalation.
- Document disposition criteria, follow‑up, and patient education before discharge.
Algorithm
- Confirm type‑1 Brugada ECG (consider high ICS lead placement).
- Screen for syncope, nocturnal agonal respirations, family SCD.
- Immediate fever control (antipyretics, cooling).
- Avoid/stop Brugada‑provoking drugs (consult up‑to‑date lists).
- Electrical storm → start isoproterenol infusion; add quinidine if recurrent.
- Risk stratify: prior VT/VF → ICD; syncope with spontaneous type‑1 ECG → consider EP study and ICD.
- Genetic counseling/testing for SCN5A and family cascade screening.
- Counsel fever protocols, drug wallet cards, and emergency plans.
- Long‑term follow‑up with EP; revisit ICD indications as phenotype evolves.
Clinical Synopsis & Reasoning
Inherited sodium‑channelopathy with coved ST elevation (type 1) in V1–V3 and risk of polymorphic VT/VF. Avoid fever and offending drugs; treat electrical storms with isoproterenol and consider quinidine. ICD is indicated for survivors of VT/VF and often for syncope with spontaneous type‑1 ECG plus inducible arrhythmias; counsel family screening.
Treatment Strategy & Disposition
Stabilize ABCs. Initiate guideline‑concordant first‑line therapy with precise dosing and continuous monitoring. Escalate to advanced or procedural interventions when predefined failure criteria are met. Define ICU, step‑down, and ward disposition triggers explicitly, and arrange specialty consultation early.
Epidemiology / Risk Factors
- Risk varies by comorbidity and precipitating factors
Investigations
| Test | Role / Rationale | Typical Findings | Notes |
|---|---|---|---|
| CBC | Anemia/leukocytosis | Context‑specific | Trend response |
| BMP | Electrolytes/renal | Derangements common | Renal dosing/monitoring |
| Condition‑specific imaging | Per topic | Diagnostic hallmark | Do not delay with red flags |
| High precordial leads (V1–V2 at 2nd intercostal) | ECG sensitivity | Unmasks type‑1 pattern | Use when suspicion high |
| Provocative drug testing | Diagnostic support | Ajmaline/flecainide challenge | Performed by EP specialists |
Pharmacology
| Medication | Mechanism | Onset | Role in Therapy | Limitations |
|---|---|---|---|---|
| Isoproterenol infusion | β‑agonist | Minutes | Suppress electrical storm | Titrate to HR; avoid ischemia |
| Quinidine | Class IA antiarrhythmic | Hours | Reduce arrhythmic events | QT prolongation; diarrhea |
| Antipyretics | COX inhibition | Hours | Suppress fever triggers | Hepatic/renal cautions |
Prognosis / Complications
- Outcome depends on timeliness of diagnosis and definitive therapy
Patient Education / Counseling
- Explain red flags, adherence, and the follow‑up plan; provide written instructions.
References
- See bibliography — Link