Killer hiding under normal oxygen saturation: a case report about methemoglobinemia

Inhaled nitric oxide (iNO) is a choice for the treatment of pulmonary hypertension (PH), especially in cases after cardiac surgery. Potential side effects include the formation of higher oxides of nitrogen and methemoglobin (MetHb). Methemoglobinemia is the oxidation of ferrous iron to iron within hemoglobin, impairing its ability to transport oxygen and resulting in tissue hypoxemia. A level of MetHb >10% will induce clinical hypoxia manifestations, and MetHb >70% may be fatal.

Herein we report a rare case of methemoglobinemia due to iNO therapy in a child after cardiac surgery. We found that as MetHb concentrations increased, pulse oximetry overestimated oxygen supplementation without warning clinicians that dangerous hypoxia was developing. Finally, MetHb and oxyhemoglobin (O₂Hb) in arterial blood gas (ABG) provide diagnostic clues. Methylene blue and low dose vitamin C (VC) were used to successfully save the life of the child.

iNO administration in the intensive care unit should be managed with close monitoring of MetHb levels during treatment. We emphasize the limitations of traditional methods used to assess oxygenation status, especially in the context of methemoglobinemia. In addition, treatment for methemoglobinemia in acute settings should be initiated as soon as possible.