Brugada Syndrome: Clinical Features, Risk Stratification, and Management.

In 1992, the Brugada brothers published a patient series of aborted sudden death, who were successfully resuscitated from ventricular fibrillation (VF). These patients had a characteristic coved ST-segment elevation in the right precordial leads on their 12-lead electrocardiogram with no apparent structural heart abnormality. This disease was referred to as "right bundle branch block, persistent ST-segment elevation, and sudden death syndrome." The term Brugada syndrome (BrS) was first coined for this new arrhythmogenic entity in 1996. BrS is more prevalent in Southeast Asian ethnic groups and was considered a familial disease due to the presence of syncope and/or sudden deaths in several members of the same family, however, the genetic alteration was only noted in 1998. The genetic characterization of BrS has proven to be challenging. The most common and well-established BrS genotype involves loss-of-function mutations in the SCN5A gene, but only represents between 15% and 30% of the diagnosed patients. Patients with BrS can present with a range of symptoms which can include syncope, seizures, and nocturnal agonal breathing due to polymorphic ventricular tachycardia or VF. If these arrhythmias are sustained, sudden cardiac death may result. Despite the significant progress on the understanding of BrS over the last two decades, there remain a number of uncertainties and challenges; we present an update review on the subject.

Idiopathic Fascicular Left Ventricular Tachycardia.

Idiopathic left fascicular ventricular tachycardia (ILFVT) is characterized by right bundle branch block morphology and left axis deviation. We report a case of idiopathic left ventricular fascicular tachycardia in a young 31-year-old male patient presenting with a narrow complex tachycardia.

The Gulf Implantable Cardioverter-defibrillator Registry: Rationale, Methodology, and Implementation.

BACKGROUND: The implantable cardioverter-defibrillator (ICD) is effective in the prevention of sudden cardiac death in high-risk patients. Little is known about ICD use in the Arabian Gulf. We designed a study to describe the characteristics and outcomes of patients receiving ICDs in the Arab Gulf region. METHODS: Gulf ICD is a prospective, multi-center, multinational, and observational study. All adult patients 18 years or older, receiving a de novo ICD implant and willing to sign a consent form will be eligible. Data on baseline characteristics, ICD indication, procedure and programing, in-hospital, and 1-year outcomes will be collected. Target enrollment is 1500 patients, which will provide adequate precision across a wide range of expected event rates. RESULTS: Fifteen centers in six countries are enrolling patients (Saudi Arabia, United Arab Emirates, Kuwait, Oman, Bahrain, and Qatar). Two-thirds of the centers have dedicated electrophysiology laboratories, and in almost all centers ICDs are implanted exclusively by electrophysiologists. Nearly three-quarters of the centers reported annual ICD implant volumes of ≤150 devices, and pulse generator replacements constitute <30% of implants in the majority of centers. Enrollment started in December 2013, and accrual rate increased as more centers entered the study reaching an average of 98 patients per month. CONCLUSIONS: Gulf ICD is the first prospective, observational, multi-center, and multinational study of the characteristics and, the outcomes of patients receiving ICDs in the Arab Gulf region. The study will provide valuable insights into the utilization of and outcomes related to ICD therapy in the Gulf region.