Brugada Sign in a Cardiac Transplant Donor

Brugada syndrome (BrS) is a rare entity represented by the Brugada sign on an electrocardiogram (EKG) and is associated with sudden cardiac death (SCD). There is little data to guide the management of donor Brugada syndrome in the setting of cardiac transplantation. A 31-year-old male sustained out-of-hospital cardiac arrest secondary to polysubstance use and was found asystole. Bystander cardiopulmonary resuscitation (CPR) with advanced cardiovascular life support (ACLS) protocol was initiated. Return of spontaneous circulation (ROSC) was achieved and the patient was taken to the emergency room (ER) in sinus rhythm with an initial presenting EKG showing the Brugada sign. A toxicological screen for cocaine was positive. The patient was eventually declared brain dead and underwent angiographic and echocardiographic evaluation as a donor heart for cardiac transplantation and was accepted for transplantation. Cardiac arrest in a young patient with a Brugada sign on EKG is a concern for BrS. Cocaine exerts a sodium channel blockade that can unmask BrS. Genetic testing for sodium voltage-gated channel alpha subunit 5 (​​​​​​SCN5A) gene mutation was negative, however, only 15% to 30% of patients carry the mutation. We proceeded with cardiac transplantation and suggested an implantable cardioverter defibrillator (ICD) for primary prevention in the recipient, should further specialized testing reveal a continued concern for BrS. We suggest the necessity for further data to guide decisions in patients with BrS undergoing cardiac transplantation.