The Brugada syndrome: is an implantable cardioverter defibrillator the only therapeutic option?

November 2002 PACE, Vol. 25, No. 11 In 1992 two Spanish cardiologist brothers, Pedro and Josep Brugada reported eight patients with aborted cardiac arrest and no demonstrable heart disease who exhibited in sinus rhythm right bundle branch block (RBBB) with prominent STsegment elevation in precordial leads V1–V3. Despite initial controversy about the diagnosis, especially concerning the possibility of a subtle arrhythmogenic right ventricular dysplasia, the repeated lack of right ventricular involvement along with consistent clinical, electrocardiographic (ECG), and electrophysiological features convinced the cardiological community that the Brugada syndrome was actually a new and important cause of sudden cardiac death (SCD) in ostensibly healthy patients.2 During the last decade an increased awareness among physicians has resulted in a growing number of patients reported worldwide. In 1998, Chen et al. were first to establish that the Brugada syndrome was a genetic disease with an autosomal dominant pattern of transmission. These investigators described mutations all affecting the cardiac sodium channel SCN5A on chromosome 3. More recently, a novel gene locus on chromosome 3, distinct from SCN5A has been identified. The genetic pattern of transmission of the disease has lead to the increased detection of asymptomatic patients affected by the disease among families of cardiac arrest survivors. Despite the major advances accomplished during the last decade, there are still unanswered issues dealing with the clinical and genetic diagnosis of the Brugada syndrome and with its management. In the September 2001 issue of the European Heart Journal appeared an article, “Task Force on Sudden Cardiac Death of the European Society of Cardiology,” to which European and American experts contributed. Since several of the leading specialists of the Brugada syndrome (namely Drs. Sylvia Priori and Pedro Brugada) were active members of this task force, the conclusions reported on the management of the syndrome have a paramount importance. The conclusion of the task force was as follows: “Some investigators supported the view that both symptomatic patients (syncopal episodes or aborted SCD) and asymptomatic patients who are inducible by programmed electrical stimulation are best managed by the implantation of an implantable cardioverter defibrillator (ICD). However, more recent data, supported the view that given the low predictive accuracy of programmed electrical stimulation, cardiac arrest survivors and patients with a history of syncope or a family history of juvenile SCD should receive an ICD. Management of asymptomatic patients is still debated and no conclusive evidence exists to guide risk stratification in this subgroup. No drug has shown efficacy in the prevention of SCD.” At the time of writing the present article (February 2002), the authors’ opinion concerning the management of the Brugada syndrome is somewhat different from that expressed in the Task Force of the European Society of Cardiology, especially regarding the role of antiarrhythmic drug therapy. This article presents the view of the current authors on the management of the Brugada syndrome including the group of symptomatic patients (cardiac arrest survivors or syncope) and the group of asymptomatic high risk patients with the Brugada-type ECG.