Brugada Syndrome: Diagnosis and Management

Introduction: The vast majority of cases of sudden cardiac arrest (SCA) are caused by ventricular tachyarrhythmias (VT), with most cases associated with structural heart disease. SCA in a structurally normal heart is an uncommon occurrence, most often occurring in the third decade of life and accounting for 5-15% of total SCA cases1-2. Common causes include Brugada syndrome, congenital long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, arrhythmogenic right ventricular cardiomyopathy, and idiopathic ventricular tachycardia1-3. The majority of these patients with a structurally normal heart are undiagnosed until presenting with sudden cardiac death. It is believed that cardiac arrest is the initial manifestation of nearly 50% of cases among those with structurally normal hearts2. Here, we review a classic case of Brugada Syndrome, a common cause of sudden cardiac arrest in young adults, its diagnostic criteria, and management in a patient presenting for elective surgery. Case Presentation: A 38-year-old male Iraq War combat veteran with a congenital cleft palate requiring multiple surgical repairs presents for cardiac evaluation when his preoperative electrocardiogram (ECG) showed new 2-3 mm coved ST elevation in leads V1 and V2 (Figure 1). On history, he reports multiple prior syncopal events while on active military service during extreme hot temperature conditions. All events were preceded by lightheadedness. He also reports a recent syncopal event 6 months prior to presentation that was preceded by palpitations causing a traumatic fall down staircases. He denies any prior cardiac workup. Review of systems was negative for chest pain, dyspnea, diaphoresis, dizziness, or flushing. Family history of cardiovascular disease was unknown as he is adopted. Social history was positive for a 20-pack-year history of cigarette use but negative for alcohol or illicit drugs. Physical exam was notable for a fistula involving the hard palate. His cardiopulmonary exam was normal. Laboratory studies included complete blood count, complete metabolic panel, thyroid stimulation hormone, urinalysis, and urine drug screen were all within normal limits. Transthoracic echocardiogram showed normal chamber size, thickness, and valves along with normal ventricular function