Intraoperative Management of a Child Undergoing Cardiac Surgery With Congenital Methemoglobinemia Detected in the...

Congenital methemoglobinemia is a rare disease that is easily overlooked in its mild form. It can lead to tissue hypoxia as methemoglobin does not possess oxygen-carrying capacity. Its management approach depends on the severity of the symptoms, methemoglobin level, and associated medical conditions. The perioperative management of congenital methemoglobinemia is well described in the literature; however, its management in children with congenital heart disease and undergoing cardiac surgery using cardiopulmonary bypass has not been reported. We present a case and its management where congenital methemoglobinemia was detected in the operating room in a child scheduled for cardiac surgery.