What about guidelines for out-of-hospital cardiac arrest with unusual cardiac anatomy?

Conventional management of out-of-hospital cardiac arrest (OOHCA) in patients with unusual cardiac anatomy may result in inferior outcomes. It is important that appropriate consideration of these patients is made when developing OOHCA resuscitation guidelines. Initiatives to improve outcomes following OOHCA, such as community training of resuscitation techniques, should take patients with unusual cardiac anatomy into account. We present a male in his twenties with heterotaxy syndrome, a congenital defect with abnormally arranged intrathoracic and intra-abdominal organs. He experienced an unwitnessed OOHCA with a downtime of approximately 20 minutes. He had complex congenital heart disease and had undergone previous palliative cardiac surgery resulting in a Fontan circulation. Despite maximal inotropic and vasopressor support, he developed multi-organ failure with diffuse hypoxic brain injury warranting care withdrawal. In this case report we discuss the important roles of appropriate community resuscitation in patients with unusual cardiac anatomy and of mechanical circulatory support in patients with single ventricle physiology.