Intravascular hemolysis secondary to aorto-atrial fistula presenting as red urine

Intravascular hemolysis is a rare but potentially life threatening cause of red urine characterized by brisk hemolysis and release of large amounts of hemoglobin into the urine. We present an unusual case of red urine in a 20-year-old male who was subsequently diagnosed with intravascular hemolysis due to an aorto-atrial fistula. Fistula formation was likely secondary to a recently implanted atrial septal occluder, which is a reported but exceedingly rare complication of the device. We discuss the diagnostic approach to hemolytic anemia and conclude with a literature review of other cases of device associated fistula formation and hemolysis.