3. It is characterized by ST segment elevation (::::::2 mm) in right pre-cordial leads (V I to V
3) followed by inverted T waves, incomplete or complete right bundle branch block, and
susceptibility to ventricular tachyarrhythmia (particularly polymorphic ventricular
tachycardia) and sudden cardiac death.
Syncope due to tachyarrhythmia usually occurs during sleep or at rest.
Typically presents in third or fourth decade.
Similar ECG patterns are seen in anterior wall infarction, arrhythmogenic right ventricular
dysplasia, acute pericarditis, Duchenne muscular dystrophy, hypercalcemia and
hyperkaliemia.
Genetically transmitted as an autosomal dominant syndrome with incomplete penetrance.
Treatment involves implantable cardioverter defibrillator (ICD) in patients who have
experienced at least one attack of cardiac arrest. Quinidine may be used if ICD
implantation is not feasible. It can also be used along with ICD to reduce number of
shocks delivered by ICD.