Brugada Syndrome is an entity in which people with no structural heart disease suffer ventricular tachycardia (VT) and sudden death. Its hallmark is an abnormal electrocardiogram (ECG) consisting of an atypical pattern of right bundle branch block (RBBB) with an unusual morphology of ST-segment elevation in leads V1 to V3. These alterations may be transitory and are often unveiled by antiarrhythmic drugs that block the sodium channel such as procainamide (PA). Recent studies have suggested that those patients with syncope or inducible VT in the baseline state at electrophysiology study (EPS) should receive an implantable cardioverter defibrillator (ICD) to improve their survival. We recently cared for 2 men whose ECG tracings demonstrated typical Brugada changes after PA infusion. Though no VT was induced in either at baseline, it was provoked in both after PA was infused.
The 2 patients underwent routine history and physical, ECG, echocardiogram, cardiac catheterization and EPS.
Patient 1 was a 51 year-old Korean man with atypical chest pain but no syncope or dizziness. Two of his 16 sibs had died (at ages 32 and 54). Patient 2 was a 38 year old Dominican man with atypical chest pain, 1 possible episode of syncope 3 months earlier and an episode of palpitations and dizziness while dancing 2 nights before admission. His mother and maternal grandmother had died between age 50 and 55. The physical exam, echocardiogram and cardiac cath data were normal in both men. At baseline their ECG demonstrated atypical RBBB and “saddle-back” type ST-segment elevation in V1-2; no VT > 5 beats was induced. After PA, ST-segment elevation became more prominent and “coved” in appearance and ventricular fibrillation was induced in both.
Procainamide may unveil heightened VT risk in Brugada patients in whom no arrhythmia is inducible at baseline EPS.
Until further data become known, we recommend ICD implantation in patients with the Brugada ECG pattern and VT that is inducible in the baseline state or after intrravenous procainamide.
C.A. McPherson, None.