A case study of Brugada syndrome.

Brugada syndrome is a little-known hereditary disease that causes severe cardiac rhythm disturbances, eventually resulting in sudden unexpected cardiac death in apparently healthy individuals. 1-3 The following is a case study of a young man diagnosed with Brugada syndrome after sustaining an unwitnessed arrest. Case Study AB is a 27-year-old man recently discharged from a longterm substance abuse rehabilitation facility with a long history of ethanol and intravenous drug use. Approximately 20 minutes after being dropped off at a restaurant, he was found unresponsive in the bathroom. EMS arrived shortly thereafter and determined the patient was in full arrest. Paramedics provided ACLS, including endotracheal intubation, CPR, and IV access. Three mg of epinephrine, 1 mg of atropine, and 6 mg of narcan were given intravenously with a return of a perfusing rhythm en route to the local hospital. The patient maintained a Glasgow Coma Scale (GCS) of 3 S/P arrest. On arrival at the community hospital, additional IV access was secured, and radiologic studies, including a head CT and chest X-ray, were obtained. An attempt to secure a right subclavian central venous line was unsuccessful. Arrangements were made to transfer this patient to a tertiary care facility by air medical transport. When the air-medical team arrived, the patient was mechanically ventilated with a V T 600 L, AC 12, 100% FiO 2 . The patient was breathing rapidly above the ventilator. His breath sounds were very course, wet, and decreased bilaterally with an O 2 sat of 86%. He was tachycardic to the 170s, diaphoretic, and normotensive with an adequate urine output. Neurologically, the patient did not respond to painful stimuli, he had a GCS of 3, and his pupils were 4 mm bilaterally and nonreactive. An EKG revealed a right bundle branch block and ST segment elevation in leads V1 to V3. The community hospital personnel reported that the patient was maintaining an oxygen saturation of 95% or better until just before the flight team arrived. They also said he had a period of hypertension that responded to a brief infusion of esmolol, which was now turned off. He had remained unresponsive, pupils fixed at 4 mm bilaterally, throughout his course there. The head CT revealed diffuse cerebral edema, decreased ventricles, and ablation of sulci consistent with an anoxic brain injury. The flight team suctioned him for copious, thick, pink, frothy secretions, and his breath sounds improved bilaterally. The patient was paralyzed adequately and sedated to control his metabolic demands and tachypnea in an effort to better ventilate and oxygenate him. He was suctioned a second time, his V T increased to 700L, and 10 of PEEP was added for improved oxygenation and ventilation in light of his profound pulmonary edema. The patient responded with increased O 2 saturation into the mid-90s and better movement of air throughout the lung fields. The patient was packaged for transport and flown to a university hospital tertiary care facility, where he was admitted to the critical care medical unit (CCMU). A 12-lead EKG was done and showed a bundle branch block and ST elevation in V1-V3—consistent Brugada syndrome. A cerebral perfusion study conducted the next day revealed no blood flow to the brain, and subsequently, life support was withdrawn.