Turk Kardiyoloji Dernegi Arsivi, vol.31, no.4, pp.219-225, 2003 (SCI-Expanded)
The Brugada syndrome is a malignant primary electrical disease of the heart caused by a defect in an ion channel gene, resulting in abnormal electrophysiologic activity in the right ventricle and characterized by(1) a phenotypic ST-segment elevation in the right-sided precordial leads often accompanied by apparent conduction block in the right ventricle, (2)grossly structurally normal heart, and(3) a propensity for life-threatening ventricular tachyarrhythmias. Due to clinical and genetic heterogeneity of the syndrome, patients with Brugada syndrome present a great challenge, both in establishing the diagnosis and determining the prognosis. Patients at risk often show periodic normalization of their ECG, leading to underestimation of incidence of the Brugada syndrome. Sodium channel blockers can be used to unmask the syndrome. The only unequivocally effective treatment to date is an implantable cardiac defibrillator, which should be considered in symptomatic and asymptomatic individuals with positive electrophysiologic testing. In this paper, we aimed to review the advances in the Brugada syndrome in connection with two asymptomatic cases with positive pharmacological stimulation tests.