Brugada Syndrome in Japan

Abstract

The incidence of Brugada syndrome (BS) is relatively high in Japan compared with the rest of the world, ranging between 0.1% and 0.2% in the general population. BS in Japan, as in other countries, is most prevalent in middle-aged men, and has characteristics ECG changes, a high recurrence rate in symptomatic patients, and relatively low incidence of SCN5A mutations. In contrast, both the incidence of a family history of BS and/or sudden cardiac death and the rate of developing cardiac events in asymptomatic patients are less in Japan than in other countries. Increased vagal tone and/or decreased sympathetic activity are suggested as provoking cardiac events. Several factors should be evaluated in risk stratification for recurrence of life-threatening arrhythmias, because there appears to be no single determinant for risk stratification: spontaneous ST elevation of coved-type (Type 1), family history of sudden cardiac death, inducible ventricular tachycardia/ventricular fibrillation and positive late potentials. An implantable cardioverter defibrillator is recommended for patients with aborted sudden cardiac death.