Propofol Infusion Syndrome With Arrhythmia, Myocardial Fat Accumulation and Cardiac Failure

Propofol is a potent and widely used central-acting sedative drug. It has been implicated in the development of a usually fatal syndrome characterized by metabolic acidosis, rhabdomyolysis, ventricular arrhythmia, and Brugada-like cardiac rhythm disturbances, all leading to cardiac and renal failure. The investigators describe a 12-year-old male patient with a fatal case of this so-called propofol infusion syndrome. Postmortem investigation showed not only the well-known myocytolysis in skeletal and cardiac muscle but also not previously demonstrated widespread fat accumulation in the myocardium. In conclusion, this cardiac fat accumulation illustrates the proposed underlying pathophysiology of impaired (muscular) free fatty acid utilization.