| Title: | CLINICAL, GENETIC, MOLECULAR, AND CELLULAR ASPECTS OF THE BRUGADA SYNDROME |
| DOI No: | 10.1142/9789812702234_0055 |
| Source: | ADVANCES IN ELECTROCARDIOLOGY 2004 (pp 203-218)
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| Author(s): | CHARLES ANTZELEVITCH
Supported by grants HL47678 from NHLBI, the American Heart Association and NYS and Florida Grand Lodges F.&A.M.
Masonic Medical Research Laboratory, 2150 Bleecker Street, Utica, NY 13501, USA
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| Abstract: | Over the past twelve years, the Brugada syndrome has progressed from being a very rare disease to one that is second only to automobile accidents as a cause of death among young adults in some countries around the world. The syndrome is characterized by an ST-segment elevation in the right precordial leads and a high risk for sudden cardiac death in young and otherwise healthy adults, and less frequently in infants and children. Patients with a spontaneously appearing Brugada ECG have a high risk for sudden arrhythmic death secondary to ventricular tachycardia/fibrillation. The electrocardiographic manifestations of the Brugada syndrome are often dynamic or concealed and may be unmasked or modulated by sodium channel blockers, a febrile state, vagotonic agents, α adrenergic agonists, β adrenergic blockers, tricyclic or tetracyclic antidepressants, first generation antihistamines, a combination of glucose and insulin, hypokalemia as well as by alcohol and cocaine toxicity. The only gene thus far linked to the Brugada syndrome is SCN5A, which encodes for the α subunit of the sodium channel. Rebalancing of ion channel currents active during phase 1 of the action potential are thought to accentuate the notch of right ventricular epicardial action potential, thus creating a transmural voltage gradient, which underlies the Brugada ECG. A further outward shift of the current leads to loss of the action potential dome, which creates the substrate and trigger for the development of VT/VF. Although an ICD is the only proven effective therapy, agents that block the transient outward current such as quinidine are being evaluated for prevention of sudden death. |
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