Objective Risk stratification for sudden death in Brugada syndrome and hence indications for implantation of an internal cardioverter defibrillator are based on the presence of a spontaneous type 1 electrocardiogram (ECG) pattern, in association with unheralded syncope or documented polymorphic ventricular tachycardia. Increasingly, awareness of sudden death in the young and the implementation of preparticipation screening programmes in sport will identify young, asymptomatic patients with the Brugada phenotype. We evaluated the predictive accuracy of symptoms, particularly unheralded syncope in victims of sudden death from Brugada syndrome.
Methods Over the past 3 years we identified 30 victims of sudden arrhythmic death secondary to Brugada syndrome. The diagnosis was based on sudden death with normal findings at postmortem and the identification of the type 1 Brugada ECG pattern in first-degree relatives (spontaneous or following an Ajmaline provocation test). All relatives underwent 12-lead ECG, echocardiography, exercise testing, 24-h Holter monitor and biochemical tests. Cardiac magnetic resonance imaging and coronary angiography were performed when appropriate.
Results Of the victims dying from Brugada syndrome, 22 (73%) were male. The mean age of sudden death was 32 years (range 8–56 years) and 90% died suddenly at rest or during sleep. We interviewed first-degree relatives and partners regarding prodromal symptoms or a history of epilepsy. Only five out of 30 victims (17%) had significant symptoms. In particular, two individuals experienced unheralded syncope, two had received a diagnosis of epilepsy and one had nocturnal seizures. Only 24% had a family history of premature sudden cardiac death. Indeed, we obtained 12-lead ECG in five patients taken before sudden death, which failed to reveal the typical Brugada ECG phenotype.
Conclusion Our results indicate that the vast majority (83%) of individuals who die from Brugada syndrome in the UK are asymptomatic and raise concerns that the absence of symptoms does not necessarily mean low risk. Better understanding of the disorder and risk stratification protocols are necessary to permit genuine reassurance in asymptomatic individuals exhibiting the Brugada phenotype.